Haploidentical peripheral-blood stem-cell transplantation for ALK-positive anaplastic large-cell lymphoma
[Show abstract] [Hide abstract] ABSTRACT: Anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL) is a rare peripheral T-cell lymphoma, accounting for approximately 3% of adult non-Hodgkin lymphomas (NHL). In this report we describe an unusual case of an ALK(+) ALCL, which presented as an aggressive mass involving upper nasal cavity and anterior skull base. The pathogenesis, histopathology with radiologic correlations, and management of this case are reviewed. A 28-year-old Asian female presented with a 3-month history of nasal congestion culminating in epistaxis. Physical examination was notable for a tissue mass obstructing nasal cavity and the sphenoid sinus. Computed tomography (CT) and magnetic resonance (MR) imaging revealed a lesion primarily involving the upper nasal cavity extending intracranially through the cribriform plates into the anterior cranial fossa. Histologic and immunohistochemical analysis of the specimen obtained through a transnasal biopsy revealed an ALK(+) ALCL. The patient underwent two cycles of chemotherapy and focal radiation therapy, achieving minimal residual disease. The patient remained neurologically unchanged with stable minimal residual disease at the 1-year follow-up. To the best of our knowledge, this is the first case of an ALK(+) ALCL that presented as an aggressive upper nasal cavity and anterior skull base lesion. This case report highlights the importance of multi-modality approaches including preoperative imaging and tissue biopsy for definitive diagnosis.0Comments 0Citations
- "An ALC below 1000/μL correlates with shorter survival and lower complete remission rate. Relapses are not uncommon (30% of cases), but often remain sensitive to chemotherapy; allogeneic bone marrow transplants may be effective in refractory cases. Radiation therapy may be necessary after completion of chemotherapy to eliminate residual sites of disease. "
[Show abstract] [Hide abstract] ABSTRACT: Non-Hodgkin lymphoma is rare in children, even though it is the third most frequent type of tumour. Management of a child with non-Hodgkin lymphoma is complex and coordinate presence of haematologists, surgeons, radiotherapists, neurologists, psychologists and other expert personnel is required. Our patient presented with a bulky mass of approximately 5 cm in diameter, which grew from his upper-left maxillary bone, thereby causing gum bulge. Thus, the left side of his face from lip to eye appeared swollen. A whole body computed tomography-positron emission tomography examination revealed that the mass was growing in the mouth and maxillary bone and that many bulky nodes were present both in the right and left neck. Histological haematoxylin and eosin assessment revealed an anaplastic large cell proliferation underlying the epithelial tissue without epithelial infiltration. The patient underwent a first cycle of chemotherapy according to the 'International Protocol of anaplastic large cell lymphoma'. The Maxillofacial Surgery Unit and the Dentistry Unit of the same hospital took care of his dental situation to avoid the spread of infective foci to the entire body. After 1 year and 3 months from the first cycle of chemotherapy, a bulky splenic mass was discovered. Laparoscopic biopsy revealed a relapse of the anaplastic lymphoma kinase-positive anaplastic large cell lymphoma in a splenic node. The patient is now alive in good conditions, and continuously followed-up by the Pediatric and Hemato-oncology Operative Unit of University Hospital of Parma. Rapidity of action and a correct multidisciplinary (oncology-maxillofacial surgery-dentistry) approach is the key to cure illness, promptly diagnose relapse and avoid the spread of infective foci from the patient's teeth during chemotherapy cycles.0Comments 0Citations
- "Worldwide , 60-70% of NHL children has a disease-free 5 year prognosis; the percentage arise to 90% in some subtypes. Recurrences (uncommon, 30%) are treated with a different protocol and often remain sensitive to chemotherapy . Allogenic or autologous marrow transplant may be taken in consideration, but it is still considered as an experimental procedure, lacking of certain longterm efficacy data. "
[Show abstract] [Hide abstract] ABSTRACT: Patients with refractory or early relapsed anaplastic large cell lymphoma (ALCL) have a poor chance of survival. We report 20 children and adolescents with high-risk relapsed or refractory ALCL who underwent allogeneic haematopoietic stem cell transplantation (HSCT). We retrospectively analysed 20 patients who relapsed between December 1991 and April 2003 during (six patients) or soon after first-line Berlin-Frankfurt-Münster-type chemotherapy (14 patients) and underwent allogeneic HSCT. Nine patients received allogeneic HSCT after the first relapse and 11 after multiple relapses. Eight patients received their transplants from matched sibling donors, eight from unrelated donors and four from haploidentical family donors. The conditioning regimen was based on total body irradiation in 15 patients. Two patients relapsed after allogeneic HSCT and died. Three patients died of transplant-related toxicity. Event-free survival at 3 years after allogeneic transplant was 75 +/- 10%. There was no influence of donor type or conditioning regimen on outcome. Two of six patients with progressive disease during frontline therapy survived compared with 13/14 patients with a first relapse after frontline therapy. Two of three patients who were transplanted with active lymphoma and all five patients who received allogeneic HSCT for relapse following autologous HSCT survived disease-free. Allogeneic HSCT is effective and has acceptable toxicity as rescue therapy for high-risk ALCL relapse. It even offers cure for patients refractory to chemotherapy, suggesting a graft-versus-ALCL effect.0Comments 54Citations
- "In very high-risk situations of progression during therapy, the time to find a donor matters. Both our results and the previous case reports show that the failure rate is not dependent on the donor suggesting that any donor can be used (Chakravarti et al, 1990; Brugieres et al, 2000; Nagler et al, 2001; Williams et al, 2002; Liso et al, 2004; Bordon et al, 2005). Even haploidentical HSCTs were successful in three of our four patients transplanted with a MMFD. "
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