Are you K. Petevi?

Claim your profile

Publications (25)51.25 Total impact

  • [Show abstract] [Hide abstract]
    ABSTRACT: Pure red cell aplasia (PRCA) is a rare cause of severe hypoplastic anemia characterized by profound depletion of erythroid precursors. Although PRCA may be associated with lymphoproliferative diseases, it has never been described in mantle cell lymphoma (MCL). We report what to our knowledge is the first case of a patient with indolent, non-nodal MCL complicated by PRCA. The patient presented with severe hypoproliferative anemia in the setting of a long-standing diagnosis of B-cell chronic lymphocytic leukemia. Bone marrow studies revealed the complete absence of erythroid progenitors. Cyclin D1 positivity on immunohistochemistry, confirmed by a positive FISH for t(11;14) (q13;q32), established the final diagnosis of MCL in conjunction with PRCA. Rituximab monotherapy led to rapid remission of splenomegaly and the leukemic picture, but the patient achieved transfusion independency only with subsequent administration of cyclosporine-A, and remained so during the subsequent 15 months despite the gradual disease recurrence.
    International journal of hematology 03/2014; · 1.17 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: A 62-year old woman was admitted in the Orthopedics Department, due to a recent right hip injury. The X-ray revealed a subtrochanteric fracture of the right femur. Her medical history was remarkable for an episode of mastitis of right breast 4 years ago, regular consumption of non steroidal anti-inflammatory drugs (NSAID) due to lumbar pain for the last 3 years, and treatment with oral bisphosphonates during the last year. Multiple osteolytic lesions and few osteosclerotic lesions were prominent in pelvic and spine X-rays (fig. 1-3). Peripheral blood smear revealed a mild leukoerythroblastic reaction and complete blood counts were as follows: Ht 24.5%, Hb 7.9 g/dL, WBC 8.59×109/L (differential count: neutrophils 86%, myelocytes 2%, metamyelocytes 3% lymphocytes 7%, monocytes 2%; few circulating erythroblasts) and platelets 209×109/L. The remaining laboratory findings revealed a highly elevated erythrocyte sedimentation rate of 120 mm/h, marked hypergammaglobulinemia (7.3g/dl; fig. 4), and hypercalcemia (10,6 g/dL). CRP was also elevated (95 mg/L) along with mild elevations in serum alkaline phosphatase (188 U/L versus upper normal limit of 106 U/L) and lactate dehydrogenase (LDH; 273 U/L versus upper normal limit of 220 U/L). Regarding the complete blood count and the blood smear there was prominent mild leukoerythroblastic reaction. A bone marrow aspiration and biopsy was diagnostic (fig. 5-7).
    Archives of Hellenic Medicne. 01/2013; 30(2):246-247.
  • [Show abstract] [Hide abstract]
    ABSTRACT: A 20 year-old male of Albanian origin with no significant past medical history was admitted at the Hematology Department with painless bilateral axillary and cervical/supraclavicular lymphadenopathy and pitting right upper limb edema. On admission his performance status was poor (4 in the ECOG scale), he was febrile (38.2 oC), normotensive, tachycardic and tachypnoic, had whooping cough and could not move his right upper limb due to edema. Symptoms began approximately 45 days before admission and gradually worsened. He reported drenching night sweats and weight loss. Physical examination revealed absence of lung sounds at the middle and lower right chest auscultation with dullness to percussion, dull cardiac sounds, right chest wall cyanosis, no hepatosplenomegaly, no head or lower limb edema and no stridor or hoarseness. The right axillary swelling was very bulky, apparently causing the posterior and anterior right chest wall, pitting upper limb, as well as right upper neck edema. Multiple, hard, painless, discrete lymph nodes up to 1.5 cm in diameter were also present in the left supraclavicular fossa and left axilla. Laboratory findings revealed leukocytosis (neutrophils: 12.1x109/L), elevated ESR (82 mm/h) and CRP (138 mg/L), marked hypoalbuminemia (22.3 g/L) and elevated LDH levels (2.3x the upper normal limit). The patient had a chest and abdomen CT scan revealing a large right axillary lymph node mass of 15 cm in diameter, infiltrating the frontal thoracic musculature up to the skin, enlarged left axillary, mediastinal and intraabdominal lymph nodes, significant right pleural effusion, small pericardial effusion and no signs of ascites. The upper limb triplex ultrasonography did not reveal signs consistent with deep vein thrombosis. The patient had a right axillary lymph node biopsy done elsewhere approximately 15 days before admission, while he was still ambulating, which had been interpreted as lymphocyte depletion Hodgkin lymphoma. However, his clinical condition rapidly deteriorated and became bedridden.After admission, a second left axillary and right cervical lymph node biopsy was performed. Lymph node imprint cytologic findings are shown in fig. 2. The right pleural effusion was drained. Pleural fluid cytology revealed large, bizarre cells, similar to those appearing at the lymph nodes, on a background of mesothelial cells (Fig. 3). On immunophenotype these cells were CD30 positive (Fig. 4), as well as CD2, CD4 positive and cytoplasmic CD3 positive (dim). Other T-cell markers (CD5, CD7 and surface CD3) were negative. A bone marrow biopsy revealed only reactive changes with no evidence of malignancy. The diagnosis was established based on lymph node biopsy morphologic and immunohistochemical findings.
    Archives of Hellenic Medicine 12/2012; 29(6):761-765.
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: A 22-year-old Albanian woman presented to our Department with left axillary and supraclavicular lymphadenopathy of 6-month duration. She experienced also bone pain and soft tissue swelling of left femur and knee. Swelling was insidious in onset and gradually increased in size. No constitutional symptoms (fever, weight loss, night sweats) were reported. Her medical history and family history were not contributory. Physical examination revealed left axillary and left supraclavicular lymphadenopathy and painful, hard swelling of lower segment of the left femur. Overlying skin appeared slightly stretched. On palpation there was no local raise in temperature and the swelling was tender. Complete blood counts, erythrocyte sedimentation rate and CRP were normal. Serum biochemistry showed slightly elevated LDH levels (230 IU/L vs. the upper normal limit of 220 IU/L). CT scan of the cervix and thorax revealed enlarged axillary and supraclavicular lymph nodes (up to 2.5 cm). CT of the abdomen was negative. A left knee X-ray was performed and showed a widened metaphysis of the femur and periosteal reaction (Fig. 1 and 2). CT of the left knee revealed a lytic lesion, that caused scalloping of the cortical region of the bone. Skeletal survey was otherwise normal. Tc-99m bone scanning revealed increased uptake on the lower segment of the left femur and the lower segment of the humerus (Fig. 3 and 4). Bone marrow aspiration and biopsy revealed only reactive changes. A left axillary lymph node biopsy was diagnostic.
    Archives of Hellenic Medicine 11/2012; 29(5-5):638-639.
  • Source
  • [Show abstract] [Hide abstract]
    ABSTRACT: Background: PET/CT negativity after ABVD is associated with favorable outcomes. However, prognostic factors for this subgroup have not been studied. Methods: Among 352 consecutive HL pts treated with ABVDx4-8, 327 responded, 287 underwent PET/CT and 229 became PET/CT(-) after ABVD. Results: Dose intensity was nearly 100%. At a median follow-up of 31 mo, 12 pts relapsed and 1 died in CR1. 95% of stage l/ll pts received radiotherapy (RT) vs. 11% advanced stage pts. Relapse free survival (RFS) was 94% and 92% at 3 and 4-6 years after the negative PET/CT. Inferior RFS was associated with advanced stage (3-year RFS 98% vs. 84% for stages l/ll vs. III/IV, p=0.001)and involvement of >5 sites (p=0.05). For the 22 stage IV PET/CT(-) pts, 3-year RFS was 70% vs. 94% (and 88% 4-year RFS) for the 39 stage ill pts. In multivariate analysis only stage III/IV was an independent prognostic factor for RFS (hazard ratio 6.6, p=0.01). At least 3/4 relapses in stages l/ll and 1/8 in stages III/IV were detectable by clinical examination; 1/8 advanced stage pts relapsed in a previously bulky site. Conclusion: A negative PET/CT after ABVDx4-6 predicts an excellent outcome in stage l/ll pts, who are typically irradiated. The majority of the relapses in this subgroup may be detectable by clinical examination only. Although not usually irradiated, PET/CT(-) stage III pts have a slightly higher relapse risk, but stage IV pts, still remain in a considerable risk (up to 30%). Therefore, although stage IV (and probably stage III) PET/CT(-) patients may require regular monitoring with imaging studies, this can probably be avoided in stage l/ll PET/CT(-) patients who have received RT.
    4th International Workshop on PET in Lymphoma, Menton 2012; 10/2012
  • [Show abstract] [Hide abstract]
    ABSTRACT: BACKGROUND: PET-2 positivity following ABVDx2 is a strong prognostic factor in advanced HL. CRP levels are usually elevated in advanced HL, but they are rapidly normalized following ABVD. AIMS: To investigate whether (1) CRP levels decline in concordance to PET-2 and (2) CRP alterations may improve the discriminative ability of PET-2. METHODS: Among 32 pts with advanced HL 30 had sequential CRP levels (abnormal if >5 mg/L). RESULTS: Among 30 pts (median initial CRP 80.4, 90% elevated), 26 were PET-2(-) and 4 PET-2(+); 3/26 PET-2(-) pts have progressed/relapsed (FN). Among 4 PET-2(+) pts, 2 continued on ABVD and progressed and 2 switched to BEACOPP-esc remaining in remission. The correlation of interim CRP with PET-2 is shown in the table:At 4-12 weeks (prior to lla,Ilia,IVa), 12-18% of patients with TN PET-2 still had abnormal CRP levels, usually due to severe obesity. CONCLUSIONS: In this preliminary analysis, persistently abnormal CRP levels were associated either with FN PET-2 or with obesity in pts with TN PET-2. PET-2 positivity was not clearly associated with CRP persistence. Further research is needed to clarify whether CRP or other biomarker serial alterations may improve the predictive capacity of PET-2.
    4th International Workshop on PET in Lymphoma, Menton 2012; 10/2012
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: An 88 year-old Greek male was admitted at the Outpatient Department of our Institution due to osseous pain. Symptoms originated nine months ago with intense pain at the right proximal antibrachial region. The pain was constant and aggraded with movements and weight lifting. Four months later, the patient visited an Orthopaedic Surgeon who proposed conservative treatment with an antibrachial cast and NSAIDs for a period of three weeks. Despite the immobilization and per os treatment, no pain relief was observed. Five months later, he was admitted at the Internal Medicine Department of a County Hospital due to fatigue. The patient was submitted to thorough clinical, laboratory and plain radiography tests that revealed a lesion at the right antibrachium as well as a pathologic ESR (115 mm/1h) and creatinine (1,9 mg/dl) and a high title of IgG antibodies. Three days later, he was instructed to perform thoracic and abdominal CT scans as well as an MRI of the antibrachium and was referred to our Department. His medical history included an acute myocardial infraction 34 years ago with an EF>60%, a prostate gland hypertophy treated conservatively and osterorthritis of the left hip treated surgically ten years ago, with total hip arthroplasty. On admission, he had no symptoms of fatigue. During clinical examination, liver and spleen were not palpable. However, intense pain was encountered while examining the right antibrachial region. The hematology profile was as follows: Hb 9.9 gr/dl, Ht 30.5%, WBC 6.59x109/L (differential count: neutrophils 89%, lymphocytes 9%, monocytes 2%) and platelets 123x109/L. The erythrocyte sedimentation rate was 100 mm/1 hour. Coagulation studies were normal. The patient had G6PD deficiency. The biochemical tests showed: BUN 112 mg/dL, creatinine 1.8 mg/dL, SGOT 11 IU/L, SGPT 6 IU/L, LDH 164 IU/L, ALP 72 IU/L, γ-GT 9 IU/L, Na+ 137 mmol/L, K+ 3.8 mmol/L, PO3— 2.9 mg/dL, Mg++ 1.7 mg/dL, Ca++ 8.8 mg/dL, serum total proteins 7.8 gr/dL, albumin 3.3 mg/dL, β2-microglobulin 6.0 mgr/dL. Serum protein electrophoresis demonstrated a spike at the area of γ-globulins. The 24h urine total protein was 1,052 gr. Bone marrow aspirate and biopsy were diagnostic.
    Arch. Hellen. Med. 08/2012; 29(4-4):511.
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: A 37-year-old man referred to the outpatient department because of prominent eosinophilia along with persistent non productive cough and recurrent sinusitis. The symptoms started 6 months ago, with concomitant discovery of an absolute eosinophil count of 2.97x109/L that remained at that level throughout this period His past medical history was insignificant. He was treated with antihistamines, corticosteroid-based bronchodilators, oral corticosteroids and several courses of antibiotics, without any improvement.On physical examination the patient appeared in no acute distress and without any remarkable signs. His full blood count was as follows: WBC=9.46x109/L (neutrophils=42%, lymphocytes=18%, monocytes=5%, eosinophils=35%), Hb=14.6 g/dL, Ht=44%, PLT=235x109/L. Eosinophils were mature in appearance, although many of them were hypogranulated with nuclear hypersegmentation. Biochemistry profile-protein electrophoresis, serum immunoglobulin levels (including IgE), ANAs ESR, CRP, serum vitamin B12 were all normal. Stool cultures for ova and parasites were also negative. Serum tryptase levels-a surrogate marker for PDGFRa rearrangement-were normal whereas chest and abdomen computed tomography weren’t indicative for the presence of malignancy or collagen vascular disease. The bone marrow aspirate showed numerous eosinophilic myelocytes and eosinophils without an increase in blasts, and the immunophenotypic studies didn’t suggest the presence of any aberrant T-cell population. The bone marrow cells were cytogenetically normal. Finally extensive evaluation of cardiorespiratory status, was completed without evidence of end organ damage. At that point a diagnostic test was performed.
    Arch. Hellen. Med. 06/2012; 29(3):379-380.
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Background: The International Prognostic Index (IPS), based on the analysis of 5023 patients treated before 1992, remains the most widely accepted prognostic system for advanced Hodgkin lymphoma. Nevertheless, IPS needs to be verified in independent patient series homogeneously treated with anthracycline-based chemotherapy (mostly ABVD) with or without radiotherapy. Few validation studies have been published so far with conflicting results.Aims: To assess the applicability of IPS in a relatively large series of patients with advanced HL under treatment with ABVD or equivalent regimens.Methods: In this retrospective study, we analyzed an homogeneous series of 510 patients with advanced Hodgkin lymphoma (stage IIB / III / IV) who were diagnosed and treated with anthracycline-containing therapy in 3 participating hospitals. Complete data for all parameters of IPS were available in 416 patients (82%), while at least half of the remaining patients were suitable for pooled analysis of the IPS (<3 versus ≥ 3 or <2 versus ≥ 2). Endpoints of the study were freedom from progression (FFP) and overall survival (OS). Results: Among 510 patients, 149 (29%) had stage IIB, 212 (42%) stage III, and 149 (29%) stage IV. The frequency of adverse IPS factors were: age ≥ 45 years 30%, male gender 57%, stage IV 30%, Hb <10.5g/dl 26%, leukocytosis ≥15x109/L 24%, severe lymphocytopenia 16% and albumin <4g/dl 65%. Only 23/416 patients (6%) had ≥5 risk factors. At a median follow up time of 81 months (6-308), 153 patients had refractory disease or relapsed and 107 had died. The 5-year FFP was 70% and the 10-year OS 73%. Among individual IPS factors, only stage IV was significantly correlated with FFP (p=0.0001), while the significance of hypoalbuminemia was marginal (0.05<0.0001 and p=0.02, respectively). Overall, the IPS was a statistically significant predictor of OS (p=0.01) but not FFP (p=0.11), as shown in the table. Instead, the discriminating ability of IPS was significantly improved for both OS (p<0.0001) and FFP (p<0.0001), when 306 patients with stage IIA were incorporated in the analysis. Summary/Conclusions: In an adequately sized series of patients with truly advanced stage HL, the IPS predicted OS and -less satisfactorily- FFP, without identifying subgroups of patients with very good or very unfavorable prognosis. Other conventional, biological or functional imaging-related factors need to be co-evaluated in order to identify sizeable subgroups of patients with sufficiently poor or favorable outcomes, suitable for treatment intensification or de-escalation respectively.
    Haematologica 06/2012; 97(s1-97 (s1)):87. · 5.94 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Autoimmune hemolytic anemia and thrombocytopenia (AIHA/AITP) frequently complicate the course of non-Hodgkin lymphomas, especially low-grade, but they are very rarely observed in Hodgkin lymphoma (HL). Consequently the frequency and the profile of patients with HL-associated AIHA/AITP have not been well defined. Among 1029 patients with HL diagnosed between 1990 and 2010, two cases of AIHA (0.19%) and three of AITP (0.29%) were identified at the presentation of disease. These patients were significantly older, and more frequently had features of advanced disease and non-nodular sclerosing histology, compared to the majority of patients, who did not have autoimmune cytopenias at diagnosis. ABVD combination chemotherapy (doxorubicin, bleomycin, vinblastine, dacarbazine) provided effective control of HL and the autoimmune condition as well. During approximately 6600 person-years of follow-up for the remaining 1024 patients, seven (0.7%) patients developed autoimmune cytopenias (three AITP, three AIHA, one autoimmune pancytopenia) for a 10- and 15-year actuarial incidence of 0.95% and 1.40%, respectively. Their features did not differ compared to the general population of adult HL. In this large series of consecutive, unselected patients, those who presented with autoimmune cytopenias had a particular demographic and disease-related profile. In contrast, patients developing autoimmune cytopenias during follow-up did not appear to differ significantly from those who did not.
    Leukemia & lymphoma 01/2012; 53(8):1481-7. · 2.61 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: A 32-year old male patient presented to our Department complaining of an asymptomatic inguino-femoral mass of approximately 9 cm maximal diameter. The lesion was painless, semi-hard to hard in texture and was not associated with any other sign of inflammation. No constitutional symptoms (fever, weight loss, night sweats), pruritus or alcohol-induced pain were reported. The patient denied any significant medical problem and was not taking any medication. There was no other abnormal physical finding. Complete blood counts, erythrocyte sedimentation rate and a full biochemical profile were absolutely normal. Whole body computed tomography did not reveal any other abnormal finding. A fine needle aspiration of the lesion was diagnostic.
    Archives of Hellenic Medicine 01/2012; 29(1-1):130-131.
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Background: Patients with HL who become PET/CT(-) after ABVD chemotherapy are expected to have a very favorable prognosis. Nevertheless, it is known that relapse risk persists for prolonged time periods. Prognostic factors determining the outcome of PET/CT(-) patients have not been adequately studied, although they may be very important for patient monitoring and counseling. Aims: The aim of this study was to identify relevant prognostic factors in this specific subgroup of HL patients. Methods: 341 patients with HL were treated with 4-8 ABVD cycles from December 2004 to mid 2011. 268 responders (CR, CRu, PR) underwent PET/CT after ABVD, while 40 did not due to technical reasons. There were missing data for 8 patients, 4 died prematurely and 21 rapidly developed progressive disease before PET/CT. Among the 268 responders who were evaluated by PET/CT, 212 patients became PET/CT(-) after ABVD, forming the patient population of the present study. Results: The median age of 212 PET/CT(-) patients was 29.5 years (15-78), 51% were males, 98% had classical HL, 26% had stage III/IV, 30% had B-symptoms, 21% had ≥5 involved sites, 37% had anemia, 15% significant leukocytosis, 9% severe lymphocytopenia, 48% had ESR ≥50 mm/h, 54% albumin <4g/dL, 20% elevated LDH and 25% high IPS (≥3). Eleven patients relapsed and 1 died in 1st CR. The median follow-up (after PET/CT) for the remaining 201 patients was 34 months (1-80). 96% of stage I/II patients received additional radiotherapy (median 3000 cGy). In contrast, 88% of advanced stage patients were not irradiated. Relapse free survival (RFS) was 98%, 96%, 94% and 92% at 1, 2, 3 and 4-6 years after the negative PET/CT scan respectively. Among the above potential predictive factors, univariate analysis demonstrated that an inferior RFS was predicted by advanced stage (3-year RFS 98% vs. 83% for stages I/II vs. III/IV, p=0.0002) and involvement of ≥5 sites (97% vs. 84%, p=0.02). Especially for the 19 stage IV PET/CT(-) patients, the 3-year RFS was only 72% vs. 89% for the 35 stage III patients. In multivariate analysis only stage III/IV was an independent prognostic factor for RFS (hazard ratio 8.8, p=0.006). At least 2/3 relapses in stages I/II and 1/8 in stages III/IV were detectable by clinical examination only; 1/8 advanced stage patients relapsed in a previously bulky site, which could have been irradiated. Summary/Conclusions: Patients with HL achieving a negative PET/CT after ABVD, have an approximately 8% cumulative risk of relapse during the subsequent 5 years. A negative PET/CT after 4-6 cycles of ABVD predicts an excellent short/mid-term outcome in patients with stages I/II, who are typically irradiated. On the contrary, stage III/IV patients rendered PET/CT(-) after 6-8 cycles of ABVD, who are not usually irradiated, still remain in a considerable risk of relapse approaching 20% within the initial 3 years. Therefore, although stage III/IV PET/CT(-) patients may require regular monitoring with imaging studies, this can probably be avoided in stage I/II PET/CT(-) patients who have received radiotherapy, in order to minimize further exposure to radiation and save health care costs.
    Haematologica 01/2012; 97(s1):87-88. · 5.94 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: BACKGROUND Essential thrombocythaemia (ET) is the most frequent myeloproliferative disorder characterized by a long natural history. The identification of JAK2V617F mutation facilitates the diagnosis and probably defines two distinct disease subgroups. AIM Aim of the present study is to report the clinical and laboratory findings, the outcome, potential prognostic factors as well as the clinical importance of JAK2V617F mutation in patients with ET. METHODS 181 patients diagnosed with ET in a single center were retrospectively studied from 1998 to 2011.RESULTS The median age of our patients was 64 years (16-91), 72 were males and 109 females. At diagnosis 75% were asymptomatic, 17% presented either a thrombotic or a bleeding episode and 11% had splenomegaly. The JAK2V617F mutation was detected in 64/95 patients (67%). The median values of Ht, Hb, WBC and PLT were 42.6% (30.8-54.7), 13.9g/dL (9.5-17.3), 8.48 x 109/L (4.44-27.4) and 781 x 109/L (468-2288) respectively. LDH levels were elevated in 25%, while the median serum erythropoietin level was 10.2IU/mL. Bone marrow biopsy revealed the presence of typical megakaryocytes in clusters in 75%, while reticulin fibrosis was found in 29% with 8 cases showing grade 2 fibrosis. An abnormal karyotype was evident in 9%. The comparison between JAK2V617F(+) and JAK2V617F(-) patients showed that JAK2V617F(+) patients presented more frequently a major event at diagnosis (p=0.05), had statistically significantly higher values of Ht (median: 44.9% vs 39.9% p<0.001), Hb (median: 14.5g/dL vs 13.1g/dL p<0.001), lower platelet counts (median: 698x109/L vs 892x109/L and lower MCV (median: 84.5fl vs 88.2fl). Antiplatelet treatment was administrated in 78%. Cytotoxic therapy was given in 80%, while the remaining patients were put under observation. The median interval from diagnosis to the initiation of cytotoxic therapy was 0.8 months (0-115). First line treatment was hydroxyurea in 86%, anagrelide in 12% and interferon-a in 1%. Complete and partial response was achieved in 68% and 26% of the patients respectively, at a median time of 1.64 months. Change of treatment was required in 11%. At a median follow up of 40 months (1.1 – 207), 94% of the patients are alive with a 5- and 10-year overall survival (OS) of 96% and 87% respectively. Events were observed in 13 patients: 6 experienced a thrombotic episode, 5 progressed to myelofibrosis and 2 developed a second malignancy. Prognostic factor analysis revealed male gender (p=0.001) and WBC counts ≥10X109/L (p=0.001) as adverse factors for OS. Bone marrow fibrosis and the presence of an event at diagnosis were of marginal significance (p=0.09). Furthermore, Hb<12g/dl and PLT<600X109/L at diagnosis correlated with a statistically significantly increased risk for the development of a thrombotic event (p=0.002 καιp=0.006, respectively), while increased LDH at diagnosis correlated with increased risk for progression to myelofibrosis (p=0.04).CONCLUSION E.T is characterized by an extremely favorable prognosis. Patients with lower hemoglobin levels and lower platelet counts present thrombotic events more frequently, while males and patients with leukocytosis are characterized by an inferior overall survival. Finally JAK2V617F(+) patients differ from JAK2V617F(-) ones regarding their hematological profile and mimic polycythaemia vera patients.
    Haematologica 01/2012; 97(s1):633. · 5.94 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: BACKGROUND Polycythaemia vera (PV) belongs to the bcr-abl negative group of myeloproliferative disorders. The introduction of the JAK2V617F mutation, has led to a clearer description of this entity. AIM Aim of the present study is to report the clinical and laboratory findings and the outcome of PV patients from a single center, as well as to evaluate the application of the new diagnostic criteria in everyday clinical practice. METHODS The WHO 2008 diagnostic criteria for PV were retrospectively applied in 61 patients diagnosed with PV between 1998 and 2011. Patients were distributed into 3 groups: Group I included patients who fulfilled both major criteria (increased Hb values for gender: Hb≥16.5g/dL in women and Hb≥18.5g/dL in men, or increased red cell mass and the presence of JAK2V617F mutation), group II included those who fulfilled the 1st major and two minor criteria (low/low-normal EPO levels and bone marrow biopsy consistent with PV). In this group JAK2V617F mutation was either unavailable or negative. Group III consisted of JAK2V617F(+) patients, who were diagnosed and treated as PV, but did not strictly fulfill the WHO 2008 criteria. RESULTS The median age of our patients was 64 years (29-91), 31 were males and 30 females. At diagnosis, 52% were asymptomatic, 21% had splenomegaly and 16% hepatomegaly. A major thrombotic or bleeding episode was reported at 30% with the most frequent being an arterial thrombosis/ischemia. JAK2V617F mutation was found in 36/38 patients (95%), median values of Ht, Hb, MCV, WBC and PLT were 53.8%, 17.3g/dL, 81.8fl, 9.050x109/L and 575x109/L respectively. LDH levels were elevated in 34%, while median serum erythropoietin levels were 6.65 IU/mL (0-28.4). Bone marrow biopsy revealed panmyelosis in 55%, fibrosis in 33% with grade 2 fibrosis in 3%, while 12% had an abnormal karyotype. First line treatment was hydroxyurea in 73%, while 24% were treated with phlebotomy only. Median time to the initiation of cytotoxic treatment was 0.9 months (0-77). Antiplatelet and anticoagulation treatment was administrated in 77% and 7% respectively. The overall response rate to cytotoxic therapy was 95% with 63% complete and 32% partial remission. Median time to response was 2.4 months. At a median follow up time of 38 months (0.9-162) 5 deaths were recorded, with a 5- and 10-year overall survival (OS) of 92% and 77% respectively. Age >65 years (p=0.029), increased LDH (p=0.026) and the presence of a thrombotic/bleeding event (p=0.048) at diagnosis were adverse prognostic factors for OS. The comparison of the 3 subgroups revealed statistically significant differences depicted in the table: Subgroup III consists of cases that cannot be objectively classified as PV or essential thrombocythaemia (ET) and might represent the pre-polycythaemic phase of PV. CONCLUSIONS Although the new diagnostic criteria of PV make the differential diagnosis between true PV and secondary erythrocytosis clear-cut, they cause difficulty in classifying patients between PV, ET and the pre-polycythaemic phase of PV. The biology of this disease subgroup is not known, while its clinical importance needs to be further elucidated.
    Haematologica 01/2012; 97(s1):639. · 5.94 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Autoimmune hemolytic anemia and thrombocytopenia (AIHA/AITP) frequently complicate the course of non-Hodgkin's lymphomas, especially low-grade, but they are very rarely observed in Hodgkin lymphoma (HL). Consequently the frequency and the profile of patients with HL-associated AIHA/AITP have not been well defined. Among 1029 HL patients diagnosed between 1990 and 2010, 2 cases of AIHA (0.19%) and 3 of AITP (0.29%) were identified at presentation of the disease. These patients were significantly older, had more frequently features of advanced disease and non-nodular sclerosing histology, compared to the majority of patients, who did not have autoimmune cytopenias at diagnosis. ABVD combination chemotherapy provided effective control of HL and the autoimmune condition as well. During approximately 6600 person-years of follow-up for the remaining 1024 patients, 7 (0.7%) patients developed autoimmune cytopenias (3 AITP, 3 AIHA, 1 autoimmune pancytopenia) for a 10- and 15-year actuarial incidence of 0.95% and 1.40% respectively. Their features did not differ compared to the general population of adult HL. In this large series of consecutive, unselected patients, those who presented with autoimmune cytopenias had a particular demographic and diseaserelated profile. In contrast, patients developing autoimmune cytopenias during follow-up did not appear to differ significantly from those who did not
    Leukemia and Lymphoma. 01/2012;
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: A 20-year-old male of Albanian origin with no significant past medical history was admitted to the Hematology Department with painless bilateral axillary and cervical/supraclavicular lymphadenopathy and pitting right upper limb edema. On admission, his performance status was poor (4 in the ECOG scale), he was febrile (38.2 o C), normotensive, tachycardic and tachypnoic, had whooping cough and could not move his right upper limb due to edema. Symptoms began approximately 45 days before admission and gradually worsened. He reported drenching night sweats and weight loss. Physical examination revealed absence of lung sounds at the middle and lower right chest auscultation with dullness to percussion, dull cardiac sounds, right chest wall cyanosis (fig. 1), no hepatosplenomegaly, no head or lower limb
    Archives of Hellenic Medicine 01/2012; 29(29):761-765761.
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Background: Patients with HL who remain PET/CT(+) after ABVD combination chemotherapy have a relatively unfavorable prognosis. Prognostic factors for the outcome of PET/CT(+) patients, especially after additional radiotherapy, have not been systematically studied. However, such factors may have a role in decision making between additional RT vs. salvage chemotherapy and autologous stem cell transplantation (ASCT).Aims: We aimed to identify predictive factors for the outcome of HL patients who remain PET/CT(+) after ABVD chemotherapy and are planned to receive additional RT.Methods: 311 patients with HL were treated with 4-8 cycles of ABVD from December 2004 to early 2011: 245 responders (CR, CRu, PR) underwent PET/CT after ABVD; 38 responders did not due to technical reasons. There were missing data for 6 patients, 3 died prematurely and 19 rapidly developed progressive disease prior to PET/CT assessment. Among 245 responders, 52 (21%) were PET/CT(+) and were retrospectively analyzed in terms of disease progression.Results: Characteristics of the 52 PET/CT(+) patients were as follows: median age 31.5 years (15-73), 58% males, 77% nodular sclerosis, 27% stage III/IV, 19% B-symptoms, 10% ≥5 involved sites, 31% anemia, 18% significant leukocytosis, 10% severe lymphocytopenia, 56% ESR ≥50 mm/h, 31% elevated LDH, 39% albumin <4g/dL, 23% IPS≥3. The median SUVmax was 5.2 (IQR 3.7-8.5) without any correlation with the above parameters. The majority of patients were irradiated (42/52 or 81%; median dose 3620 cGy; range 2880-4600). Ten (10) patients were not irradiated (too extensive disease 2, rapid progression 1, medical decision 1, refusal 1, findings judged borderline by treating physician 5). After a median follow-up of 29 months (2-67), 22/52 patients have experienced disease progression. The median SUVmax was 6.7 versus 4.1 for patients with and without disease progression respectively (p=0.02). Progression free survival (PFS) was 73%, 63%, 51% and 46% at 1, 2, 3 and 4-5 years after PET/CT respectively. The 3-year PFS was 59% vs. 24% for patients with SUVmax < and ≥9 respectively (p=0.0005). Among other factors, only anemia significantly affected PFS in univariate analysis (p=0.01). In multivariate analysis, SUVmax ≥9 was the only independent prognostic factor for PFS (hazard ratio 3.1; 95% CI: 1.1-9.2; p=0.037) obscuring the significance of anemia (p=0.17). The significance of SUVmax ≥9 was maintained, if the analysis was restricted to the 42 irradiated patients (3-year PFS 69% vs. 30%, p=0.004). In 14 of the irradiated patients, a repeated PET/CT scan converted to negative: only 2/14 have relapsed for a 3-year PFS of 83%.Summary/Conclusions: Despite additional radiotherapy, patients with HL who remain PET/CT(+) after ABVD have a 50-60% risk of relapse over the next 4 years. Patients with lower SUVmax (optimal cut-off to be determined) may benefit more from additional radiotherapy, while more intense uptake was associated with an even higher risk of disease progression. These findings may facilitate the selection of optimal treatment in this patient subgroup with uncertain prognosis. The potential contribution of conventional prognostic parameters needs further investigation. Patients rendered PET/CT(-) after RT appear to be at lower risk of relapse.
    Haematologica 01/2012; 97(s1):562. · 5.94 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Background: PET/CT is crucial in the post-treatment evaluation of patients with HL, while several studies support the implication of interim PET/CT in the design of treatment strategy. Although baseline PET/CT is recommended, it is not considered mandatory for the initial staging of HL. Furthermore, the effect of baseline PET/CT on the choice of the first-line treatment has not been systematically studied so far. Aim: To compare the results of conventional clinical staging (CS) and PET-based staging (PET-S) in patients with previously untreated HL.Methods: Sixty-seven patients with HL, who were selected based solely on the availability of baseline PET/CT results, were evaluated by conventional and PET-based methods. Results: The median age of the 67 patients was 33 years; 40/67 were males. Based on CS, 8, 25, 17 and 17 patients had stage I, II, III and IV. The median number of involved anatomic sites was 1, 2 (1-5), 5 (2-14) and 7 (4-11) for patients with CS I, II, III and IV respectively. Based on PET/CT results, the corresponding figures were 2 (1-5), 3 (2-9), 7 (2-14) and 11 (5-14). The number of involved anatomic sites as determined by CS or PET-S were highly correlated (Spearman’s rho 0.80). Compared to CS, PET-S resulted in stage change in 20/67 patients (30%): 17 were upstaged (25%) and 3 were downstaged (5%), as shown in the table. Similarly, PET-S revealed more sites of involvement than CS in 50/67 patients (67%). Among 20 patients with potential stage shift, major treatment modifications could have been justified in 16 (24% of the total) based on PET-S. However, in only 7/16 a different therapeutic strategy was actually applied due to PET/CT findings. Among 33 patients with CS I/II, 21 (64%) actually received wider field irradiation, based on the identification of more involved sites by PET/CT. With respect to bone marrow (BM) findings, 43 patients had no evidence of bone/BM uptake and all of them had negative BM biopsies. A diffuse BM uptake was observed in 11 patients: 2/11 (18%) had histologically proven BM involvement. Multifocal bone/BM uptake was noted in 13 patients: 5/13 (38%) had histologically proven BM involvement. Conclusions: PET-S resulted in the identification of more disease sites in the majority of patients with HL. A stage shift was noted in 30% of patients, with the majority of them being upstaged. In everyday practice only 10% of the patients had their treatment strategy changed due to PET/CT findings, although less radical changes did occur in 2/3 of early stage patients due to the widening of radiotherapy fields. The variable use of PET/CT for the initial staging of HL and its heterogenous effect on treatment decisions may render comparisons of retrospective series difficult. Furthermore, PET/CT-based design of radiotherapy may lead to the use of larger fields, in an era that minimization of radiotherapy is warranted. The optimal use of baseline PET/CT in HL needs to be further evaluated.
    Haematologica 01/2012; 97(s1):88-89. · 5.94 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: BACKROUND: The optimal treatment of very elderly patients with DLBCL has not been been well established. Many patients have received chemotherapy (CIT) without anthracyclines with rather poor results. The combination of Rituximab with CHOP has greatly improved the prognosis of DLBCL. The administration of Rituximab in very elderly patients might permit dose reductions in the chemotherapy regimen without significant loss in efficacy. AIMS: To describe the clinical characteristics, the actually delivered doses of chemoimmunotherapy (CIT) drugs in routine clinical practice and the final outcome of very elderly patients with DLBCL in the era of CIT. PATIENTS AND METHODS: Among 579 patients with DLBCL treated in 5 medical Centers in the era of CIT, 56 were older than 80 years (10%). We studied the clinical and laboratory characteristics of these patients (individual factors of IPI, gender, Β-symptoms, anemia, lymphocytopenia, albumin), Progression Free Survival (PFS) and Overall Survival (OS) in comparison with 523 patients younger than 80 years, who were treated during the same period. In addition, we analyzed the relative dose intensity (RDI) of the 4 drugs (except of corticosteroids), which was actually delivered to the very elderly patients, using as reference the doses and time intervals of standard R-CHOP-21. RESULTS: The median age of the very elderly patients was 82.5 years (80-91). The frequency of adverse prognostic factors (IPI, R-IPI, low serum albumin, severe lymphocytopenia) was significantly higher in very elderly patients, but the difference was mainly due to the favorable prognostic profile of the younger patient group (<60 years). In contrast, the characteristics of patients aged 61-79 and ≥80 years were similar. The 5-year PFS was 81%, 68% and 62% (p=0.0005) in the group of ≤60, 61-79 and ≥80 year-old patients respectively (p=0.12 for 61-79 vs. ≥80). The corresponding 5-year OS was 90%, 65% and 55% (p=0.0001, but p=0.048 for 61-79 vs. ≥80). In multivariate analysis of PFS, age ≥80 years had no independent prognostic significance when IPI and lyphocytopenia were taken into account. On the contrary, it was an independent prognostic factor for OS with a relative risk 2.9 (p<0.001) and 2.1 (p=0.01) compared to patients <80 years or 61-79 years respectively. Among patients ≥80 years-old, who received >1 cycle of anthracycline-based CIT, the median RDI and IQR were: Rituximab 85% (78-99), cyclophosphamide 76% (67-86), anthracycline 60% (50-69), vincristine 64% (50-76). Moreover, 6/56 patients did not receive anthracyclines and 2 received only 1 cycle. SUMMARY/CONCLUSIONS: CIT provides prolonged survival in >50% of DLBCL patients ≥80 years-old. These results are very encouraging even though RDI for chemotherapeutic drugs was low in the everyday clinical practice. Age ≥80 years was not an independent prognostic factor for PFS but only for OS. These observations suggest that the majority of very elderly patients with DLBCL should not be treated with palliative approaches. Instead they should receive as complete R-CHOP-like CIT as possible with curative intent.
    Haematologica 01/2011; 96:438. · 5.94 Impact Factor