CD4+ CD56+ lymphomas are recently described rare hematological neoplasms thought to be originating from plasmacytoid dendritic cells. Clinical features typically include cutaneous nodules associated with lymphadenopathy and/or splenomegaly and bone marrow involvement during the disease course. Complete remission (CR) is frequently obtained (around 80%) with polychemotherapy. However, median time to relapse is about 9 (range 3–18) months and median survival is only 13 months. Age > 60 years is a poor prognostic factor. Long term survival has only been reported following allogeneic SCT (

Bone Marrow Transplantation 32, 637–646, 2003
;
Blood 99,1556–1563, 2002
). We report a case of CD4+ CD56 + lymphoma with prolonged survival after an autologous PBSCT. A 71-year-old male presented in the fall of 2001 with facial erythema that progressed to several purple cutaneous nodules also involving his trunk. Skin biopsy done in June 2002 confirmed the diagnosis of CD4+ CD56+ lymphoma of the skin. Staging studies including CT scan of the chest, abdomen and pelvis, and bone marrow aspiration and biopsy were negative. The patient received chemotherapy with CHOPE (cytoxan 750 mg/m2, adriamycin 50 mg/m2, vincristine 1.4 mg/2, prednisone 100mg x 5 days, etoposide 120 mg/m2) for six cycles starting June 2002, and achieved a CR with disappearance of all skin lesions. CT scan of the chest pretransplant revealed a single 2 cm paratracheal lymph node that was also PET positive. Stem cells were collected using cytoxan 4 gms/m2 followed by 10 mcg/kg/day of G-CSF. The patient underwent autologous stem cell transplantation in February 2003 using BEAM (carmustine 300mg/m2 day −6, etopside 200mg/2 days −5 to −2, cytarabine 400mg/m2 days −5 to −2, melphalan 140 mg/m2 day -1) as a preparative regimen. Neutrophil engraftment occurred on day 10 and platelets engrafted on day 14 post SCT. The paratracheal lymph node was still visible on the PET scan 6 months post SCT. However, restaging studies at one year and beyond have all been negative for disease reoccurrence including a CT chest that revealed a calcified paratracheal lymph node that was negative on PET scan. The patient continues to be in complete remission 37 months from diagnosis and 30 months post SCT. The aggressive nature of CD4+ CD56+ lymphoma is evident by its 9-month median time to relapse and short survival. Therefore, aggressive treatment protocols incorporating stem cell transplant should be utilized in their management and should not be limited to only younger patients. As illustrated by our patient, the use of autologous stem cell transplant in the elderly can be done safely with encouraging results.

Topics:
cd56 antigens, lymphoma, neural cell adhesion molecules, older adult, peripheral blood stem cell transplantation, skin, positron-emission tomography, autologous stem cell transplant, complete remission, computed tomography
2005, The American Society of Hematology
2005
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