In August of 2000, a 18-yr-old male was admitted with a 1-mo history of fever and multiple subcutaneous nodules on the trunk and extremities. There were no other notable findings on physical examination. Imaging studies including chest X-ray, computed tomography (CT) scan of abdomen, and brain magnetic resonance imaging (MRI) scans were performed for fever of unknown origin (FUO), and there was no involvement of other organs except splenomegaly. Complete blood count (CBC) findings were consistent with mild anemia and leukopenia (Hb level, 10.2 g/dL; leukocyte count, 1.28×109/L; and platelet count, 164×109/L). Biochemical tests revealed elevated levels of aspartate aminotransferase (AST; 73 IU/L), alanine aminotransferase (ALT; 59 IU/L), and lactate dehydrogenase (LDH; 752 IU/L), and ferritin >1,000 µg/L.
Immunohistochemical staining was performed on a biopsy specimen from a skin nodule on the leg, which showed atypical lymphocyte infiltrations in a lobular panniculitis pattern (). The atypical lymphocytes were positive for CD3 () and CD45RO and negative for CD20 and CD56. There was fat rimming () with CD8-positive atypical lymphocytes (). An analysis of TCR gamma gene rearrangement using the skin specimen was negative. This diagnosis was SPTL. Bone marrow aspiration showed hemophagocytic histiocytes. There was no evidence of bone marrow involvement of the lymphoma.
Fig. 1 Biopsy of a skin nodule. (A) The skin tissue showed dense lymphocytic infiltrate in lobular panniculitis-like pattern (arrowheads) with focal dermal infiltrations (arrow) (hematoxylin and eosin stain, ×40 magnification). (B) The infiltrated lymphocytes (more ...)
This patient was treated with a COPBLAM-V (cyclophosphamide/vincristine/prednisolone/bleomycin/doxorubicin) regimen. After 1 cycle of chemotherapy, fever, leukopenia, and nodules disappeared. After 4 cycles of the chemotherapy, the patient declined further chemotherapy, and did not return to the clinic. One year later, he returned with relapsed fever and skin rash with nodules. He showed pancytopenia (Hb level, 9.7 g/dL; leukocyte count, 0.72×109/L; and platelet count, 84×109/L). He was treated with modified CHOP (cyclophosphamide/doxorubicin/vincristine/prednisolone) regimen for 4 mo. The skin nodules persisted. Re-biopsy of the skin indicated that SPTL remained. The disease was thought to be refractory to chemotherapy.
Treatment with CsA 200 mg/d was attempted, and the patient achieved remission after 1 mo. The treatment was maintained for 12 wk, and then the dose was slowly tapered for 6 wk. As a result, a complete remission (CR) was achieved. For the next 4 yr, no recurrences were observed. Five years after the achievement of CR, the patient presented with palpable masses in posterior neck, chest wall, back, and abdomen. Whole body PET-CT scan and CT scan of the abdomen revealed multiple metastatic lesions (). Laboratory findings showed leukopenia (3.1×109/L), abnormal liver function (AST/ALT (IU/L): 75/102), and elevated LDH (436 IU/L). After CsA 400 mg/d for 4 d during a 1-mo period, no response was noted. Therefore, cisplatin/cytarabine combination chemotherapy was administered up to 2 cycles, but the skin lesions remained. Finally, he was treated with CsA 800 mg/d for 7 d. When he returned to the clinic, the lesions markedly improved. PET-CT scan showed no definite evidence of disease (). We considered the disease status as CR, and continued CsA at 400 mg/d over 10 wk. There has been no evidence of recurrence 3 yr after retreatment.
Fig. 2 Whole body PET-CT (A) At the second relapse, multiple nodules with high signal intensities were noted in multiple subcutaneous and muscular areas. (B) After treatment with CsA, no active lesions were seen in the PET-CT scan. Abbreviations: PET-CT, positron (more ...)