[show abstract][hide abstract] ABSTRACT: A 79-year-old woman suffering from seronegative RA for 3 years (established according the 2010 American College of Rheumatology/European League Against Rheumatism classification criteria), with erosions in the proximal interphalangeal joints and receiving methotrexate 10 mg/wk for approximately 1 year, which was well tolerated, was admitted to our hospital, complaining of fatigue and weakness. Her symptoms had started a year before and were gradually getting worse. During the previous 6 months, the RA was in remission (according the 1981 preliminary criteria for clinical remission of RA), and the patient continued to receive the same dose of methotrexate. She had no history of diarrhea, traveling abroad, or exposure to contagious diseases. On physical examination, the patient was pale; her spleen and liver were palpable approximately 2 to 3 cm below the left and right costal margin, respectively; and an ejection-systolic flow murmur was auscultated throughout her chest; there was no peripheral lymphadenopathy, fever, or skin rash. Laboratory tests showed leukopenia (2300/µL), lymphopenia (650/µL), normocytic anemia (hematocrit, 25.8%; hemoglobin, 8.7 g/dL), high erythrocyte sedimentation rate (133 mm/hr), high C-reactive protein (25.1 mg/L; reference range, 0–6 mg/L), high plasma fibrinogen (436 mg/dL), low serum iron and total iron-binding capacity (32 and 191 µg/dL, respectively), and polyclonal hypergammaglobulinemia. Chest radiograph did not show abnormalities. An abdominal ultrasound showed liver and spleen measuring at the upper limit of normal (13 and 13.5 cm, respectively). However, computed tomography of the abdomen showed hepatomegaly (16.8 cm) and splenomegaly (16 cm). Rheumatoid factor, antinuclear antibodies, C3, C4, antimitochondrial antibodies, hepatitis B surface antigen (HBsAg), HBsAb, HBcAb, anti–hepatitis C virus, Mantoux test, and peripheral blood smear for Plasmodium and Trypanosoma were negative. Serum calcium, glucose, total and direct bilirubin, lactate dehydrogenase, haptoglobulin, and reticulocyte count were within normal limits. However, indirect immunofluorescence antibody testing was strongly positive for Leishmania (1/800), and bone marrow aspiration revealed abundant Leishmania parasites. Intravenous treatment with liposomal amphotericin B (1.5 mg/kg) was administered for 21 days. On the seventh day, the inflammatory markers started to return to normal values. After the fifth infusion of amphotericin, the serum creatinine levels increased to 1.6 mg/dL, which gradually decreased to 1.2 mg/dL over the following 10 days. Six months later, the patient is in good condition, without any symptoms or signs of visceral leishmaniasis relapse.
Journal of clinical rheumatology: practical reports on rheumatic & musculoskeletal diseases 01/2012; 18(1):59. · 1.19 Impact Factor
[show abstract][hide abstract] ABSTRACT: A 31 year-old woman with a medical history of metastatic malignant disease involving the liver and the lungs, was admitted in our clinic due to malaise and fatigue that were rapidly deteriorated. Physical examination revealed skin and conjunctival pallor and sinus tachycardia, as well as an enlarged, nodular, hard, non-tender liver, without splenomegaly. The complete blood count showed a normochromic, normocytic anemia, leucopenia and a profound thrombocytopenia (Table 1). Examination of the peripheral blood smear confirmed the results of the complete blood count and showed a slight left shift of the white blood cells. The biochemical profile was notable for a pronounced increase of the serum LDH and ferritin (Table 1). A bone marrow aspiration was performed. Pictures 1 to 4 show the bone marrow smear of the patient at high magnification.
[show abstract][hide abstract] ABSTRACT: The case is reported of a 79 year-old female with rheumatoid arthritis, receiving treatment with methotrexate, who presented with hepatomegaly, splenomegaly, leukopenia and hypergammaglobulinemia. The diagnosis of visceral leishmaniasis was established and the patient had an excellent outcome. Visceral leishmaniasis is an extremely rare theraoccurrence in patients with rheumatoid arthritis treated with methotrexate. Other than visceral leishmaniasis reports of many other opportunistic infections or complications in patients treated with methotrexate for rheumatoid arthritis were found in the literature.
[show abstract][hide abstract] ABSTRACT: A 69-year-old woman presented with fatigue, mainly in exercise and an erythematous papular rash, which had appeared on her trunk one month before her admission and increased gradually. Her medical history included hypertension, hyperlipidemia and coronary disease. On admission, her temperature was 36.8 oC and her blood pressure was 150/90 mmHg under amiloride and hydrochlorothiazide treatment in combination with metoprolol tartrate administration. The physical examination revealed pallor and skin lesions, 5-25 mm in diameter that were not painful, tender or pruritic, and were spread over the trunk. Other physical and neurological examinations showed no abnormalities. The respiratory and heart sounds were clear. Laboratory tests indicated anemia, with a hemoglobin level of 9.5 g/dL, leukocyte count of 12.6x109/L (differential count: neutrophils 36%, lymphocytes 40%, monocytes 12%, eosinophils 1% and blasts of monocytic morphology 11%), and thrombocytopenia (88x109/L). Lactate dehydrogenase (715 IU/L) and uric acid (8.1 mg/dL) were increased, while there were no other abnormalities in the biochemical profile of the patients. The bone marrow aspiration showed an infiltration by blasts (55%) that were peroxidise positive. The marrow blasts displayed positive immunophenotyping for CD33, CD13, CD15, CD41a, CD56, CD64, CD68, CD117 and HLA-DR. We performed a skin biopsy which also revealed infiltration in the dermis and subcutaneous fat tissue by leukemic cells that were positive for CD33, CD15, CD45, CD56, CD68 and HLA-DR. Chromosome analysis was normal. Based on the above laboratory and immunophenotypic characteristics the diagnosis was established and the patients started the appropriate therapy. The skin lesions disappeared and became pigmented after two courses of this therapy, and the patient achieved CR. He remained well, with no evidence of relapse nine months after his diagnosis. However, 4 months post therapy initiation, fine, transverse depressed lines that were parallel and evenly spaced developed on all of her fingernails).
[show abstract][hide abstract] ABSTRACT: A 66-year-old man was admitted to the hospital due to afternoon fever up to 38.5 C, fatigue, blurred vision and diplopia, which was started the day before admission. His medical history was unremarkable and included only mild hypertension and hypercholesterolemia under treatment with atorvastatin. The physical examination revealed limited motility of the right eye in all directions. Pupil reflexes were normal. Ophthalmologic examination confirmed the diplopia. The laboratory profile revealed anemia (Hb 9.8 g/dL) with normal white blood cell and platelet counts, hypoalbuminemia (2.8g/dL), high 2-microglobulin (4.6 mg/L), IgA paraprotein (2.370 mg/L), and increased serum free -light chain (6.820 mg/L). ESR was 82mm/1h and CRP was negative. There was anosoparesis but renal function and LDH levels were normal. Common or opportunistic infections were excluded by blood and urine cultures and CT scans of the thorax and abdomen. Due to the presence of ΙgAκ paraproteinemia, a bone marrow aspiration was performed and the trephine biopsy showed the presence of plasma cell infiltration of the bone marrow at a proportion of 55%. Many of them were atypical, with large multilobulated nucleus. Both conventional cytogenetics and FISH analysis revealed no abnormalities. MRI of the spinal cord and plain radiography of the skeleton were also normal. An MRI of the orbits revealed a soft-tissue mass infiltrating the left carvenous sinus. A mass biopsy was performed and he initial pathologic evaluation revealed a high grade pleomorphic neoplasm that failed to express multiple epithelial, mesenchymal, lymphoid and melanoma immunohistochemical markers. Subsequent fresh tissue evaluation with touch imprints and immunophenotypic characterization confirmed the plasma cell origin of the tumor. Thorough retrospective review of the touch imprint smears clearly showed the plasmacytic cytologic features. The diagnosis was established and the patient was treated with systemic therapy and also local radiotherapy in the mass of the nervous system. After two cycles of treatment, there was a dramatic deterioration of the disease with leukemic phase which could not respond to every therapy that was used. The patient was died within 4 months from initial diagnosis.
[show abstract][hide abstract] ABSTRACT: A 43-year-old woman presenting with confusion, ane¬mia, lymphopenia, thrombocytopenia and cervical microlymphadenopathy was admitted to the Hospital after complaining for 20 days of fever, headache and rachialgia. The past medical history has included a chronic diar¬rhoea syndrome for the last 5 months, which was due to non-specific colitis. Physical examination revealed pallor, generalized microlymphadenopathy, mild splenomegaly (2 cm below left costal margin), and confusion. The laboratory findings included normochromic/normocytic anemia (Hb 9.6 g/dL), mild leukocytosis (11x109/L) with severe lymphopenia (0.2x109/L), and thrombocytopenia (56x109/L). Peripheral blood evaluation showed the presence of a small propor¬tion of blast cells (<1%). The investigation for rheumatoid disorders, thyroid, liver and renal function was normal. Chest X-rays was within normal limits. Abdomen CT scan confirmed mild splenomegaly but showed no lymphadenopathy. A bone marrow aspiration showed an infiltration by myeloblasts at a proportion of 45%. Conventional cytogenetic analysis revealed a 46, XY, del(16)(q22) karyotype. Immunophenotyping showed positivity of blastic cells for myeloperoxidase, CD13, CD15, CD33. Patient was positive for HIV antibodies. OKT4 lymphocytes were 6%. A lumbar puncture was performed and cerebrospinal fluid (CSF) was found to have a cell count of 500/μL, hypoglicorrachia (18 mg/dL), and increased protein levels. CT and MRI scans of the brain showed multiple con¬trast-enhancing lesions in the basal ganglia and multiple lesions with ring-enhancement over the cerebellum, frontal and temporal lobes. During antifungal treatment left hemiparesis appeared. A new brain CT showed a hypodense lesion in the posterior brachium of the left inner capsule; at its base the lesion showed a hypodense streak coherent with thromboembolic damage. The antimycotic treatment entirely cured the hemiparesis and normalized the CSF. However, patient died 2 months later during therapy for hematological malignancy.
[show abstract][hide abstract] ABSTRACT: A 59-year old male presented in our Outpatient Department with a 10-day history of purpura and a 24-hour history of gum bleeding. The patient also complained for fatigue during the last month. There was a history of myelodysplasia in his father and of chronic lymphocytic leukemia in his grandmother.The physical examination revealed pallor, purpura in the lower extremities and signs of bleeding in his gums. The full blood count revealed hemoglobin of 9.1g/dL, a white cell count of 34x109/ L, neutrophil count of 0.7x109/L and a platelet count of 7x1012/L. Peripheral blood smears revealed the presence of abnormal cells with basophilic granules [fig. 1]. Other laboratory testing was remarkable for a serum urea of 55 mg/dl, creatinine 2.1 mg/dl and LDH 2160 U/dl. Chest X-rays revealed no abnormalities. The bone marrow aspirate was grossly hypercellular with normal hematopoiesis replaced by a population of abnormal cells, accounting for 65% of nucleated cells and eosinophils, and their precursors, accounting for 22% of nucleated cells [fig. 2]. No Auer rods were seen although some abnormal cells contained fine granules. Cytochemically, these cells were positive for Sudan black and chloracetate esterase and 27% of nucleated marrow cells showed weak staining with non-specific esterase. Immunophenotyping showed positivity for myeloperoxidase, CD33, CD34, HLA-DR, CD117, CD13, CD64 and CD15, and B and T cell markers were negative. Cytogenetic analysis of bone marrow revealed 46,XY,t(16;16)(p13;q22) in all 20 metaphase cells. The diagnosis was made and the patient received appropriate induction chemotherapy with posaconazole prophylaxis.On day 16 of induction therapy, he became febrile and was started on empirical ceftazidime and amikacin according to creatinine clearance, as he was neutropenic (neutrophils 0.1x109/L). The chest X-ray showed a dense infiltrate in the right lower lobe [fig. 3]. Vancomycin and voriconazole were added two days later as the fever had not been resolved and the patient remained in severe neutropenia. The thoracic high resolution computed tomography (HRCT) performed on d5 of the fever and showed also a cavitating infiltrate with halo sign in the left lower lobe. The CT was highly suspicious of invasive aspergillosis and the patient was started on a combination therapy consisting of amphotericin B 1.5 mg/ kg i.v. q.d. and voriconazole 4 mg/kg i.v. q12 h. Ceftazidime was replaced by imipenem and vancomycin continued. A bronchoalveolar lavage failed to document an active infection with negative Gram, Grocott and acid-fast stains as well as negative cultures, but there was a possitive polymerase chain reaction for Aspergillus. Follow-up CT scans (on d15 and 40) showed a reduction of the cavitary infiltrate. The patient was without fever from the 5th day of the antifungal combination therapy.
[show abstract][hide abstract] ABSTRACT: An 18-year-old woman presented to the outpatient clinic because of fever, chills, malaise and symptoms from the upper respiratory and gastrointestinal tract. Five days before her admission she had sore throat, cough and fever up to 38 oC. She was treated with amoxicillin without any improvement. Subsequently, abdominal pain with vomiting and diarrhea, weakness and dyspnea on slight exertion were added; fever was up to 39 oC, and thus the patient came to the hospital. Her past medical history was unremarkable. Physical examination on admission, revealed palor a faint, maculopapular rash in the extremities, cervical and supraclavicular microlymphadenopathy (smaller than 0.5 cm in diameter, painless and mobile) and a mild non-tender splenomegaly (3 cm below costal margin). The liver was not palpable. Patient’s temperature was 38.2 oC, while her pulse rate was 98/min and the respiratory rate was 26/min. Fundoscopy and neurological examination were unremarkable. Her haematological profile was as follows: Ht 18.6%, Hb 6.5 g/dL, MCV 62 fL, MCH 21.6 pg, reticulocytes 0.1%, white blood cell counts 3.6x109/L (neutrophils 48%, lymphocytes 44%, monocytes 7%, eosinophils 1%) and platelet counts 158x109/L. The morphology of the peripheral blood smear is shown in Figure. Serum biochemistry was as follows: urea 0.18 mg/dl, creatinine 1.2 mg/dL, total bilirubin 0.80 mg/dL, SGOT 35 IU/L, SGPT 40 IU/L, alkaline phosphatase 128 IU/L, γ-GT 25 IU/L, LDH 290 IU/L, uric acid 5.2 mg/dL and total proteins 6.1 g/dL with normal electrophoretic diagram. The bone marrow aspiration findings are shown in Figure 2. Symptomatic therapy was administered. Fever and other symptoms disappeared after three days, while reticulocytosis and a mild leukocytosis were observed. Two weeks after the initiation of fever, the patient had no symptoms but anemia persisted. As hypochromia, microcytosis and other significant morphologic alterations of red cells were observed, electrophoresis of hemoglobin was performed but no increase of HbA2 was found. Red cell survival was slightly reduced (19 days) and the osmotic fragility was decreased [The mean corpuscular fragility (MCF) is obtained as 3.45 g/L of sodium chloride, when the normal range is 4.0-4.45 g/L.].