May 2004

Fig.4

Fig.5

Fig.6

Diagnosis: Subcutaneous panniculitis-like T-cell lymphoma (with loss of T-cell markers and expression of the NK marker CD56) with features of cytophagic histiocytic panniculitis.

Histopathology:A surgical biopsy of a subcutaneous nodule on the right buttock was obtained and revealed subcutaneous neoplastic infiltrate with angiodestructive features. Fig. 4 shows a panniculitis with granulomatous features; a mixed inflammatory infiltrate is present In Fig. 5 "bean bag cells" (macrophages with erythrocyte, lymphocyte, and karyorrhectic debris) are seen. Fig.6 is a high power view of the panniculitis showing atypical lymphocytes. Further analysis revealed a clonal population with T-cell receptor rearrangement and CD2/3/56+.

Hospital Course:
The patient was admitted to BJH and additional studies were performed including skin biopsy and bone marrow biopsy. Skin biopsy of a lesion on the patient's right arm revealed epidermal atrophy with focal ulceration and interface dermatitis. No evidence of lymphoma or panniculitis. Bone marrow biopsy revealed slightly decreased cellularity at 40%, dysmyelopoiesis, and hemophagocytosis. Flow cytometry did not reveal clonality, but did detect an increased percentage of cells expressing NK cell marker CD16/56+.

Additional laboratory tests were consistent with hemophagocytic syndrome with an elevated ferritin of 18,614 and elevated triglycerides. Due to the strong correlation of hemophagocytic syndrome with lymphoma and viral infection, additional tests were performed: EBV/CMV/HTLV/HIV serologies were negative; ANA/ENA/ANCA/Rheumatoid factor/RPR serologies were negative; LDH was 1,075. Positron emission tomography revealed numerous subcutaneous areas of increased FDG uptake, the largest of which was located on the right buttock.

The patient received CHOP chemotherapy for several months. He recently expired from complications of hemophagocytic syndrome.

This case waas presented by Dr. Dustin James and Dr. Helen Kim-James.