Hemophagocytosis and coagulopathy associated with cutaneous γ‐δ T‐cell lymphoma

Abstract

A 34-year-old Caucasian woman was admitted with an 8-month history of a skin rash and new-onset coagulopathy. Her complete blood count on admission revealed a WBC count of 4,700 per mL, a Hb level of 9.1 g per dL, and a PLT count of 47,000 per mm 3 . A skin biopsy was consistent with a cutaneous gd T-cell lymphoma. A marrow aspirate smear revealed marked hemophagocytosis, showing mature macrophages with engulfed RBCs (A), erythroblasts (B), PLTs (C), and MNCs (D). The prothrombin time (29.2 sec) and partial thromboplastin time (66.6 sec) were prolonged, fibrinogen was depressed (<30 mg/dL), and ferritin (46,610 mg/mL) and lactate dehydrogenase (LDH 2886 u/L) were elevated. Combination chemotherapy with etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (DA-EPOCH), plus alemtuzumab (a monoclonal antibody to CD52 antigen present on T lymphocytes), was administered from Hospital Days 2 through 7 under an institutional review board-approved protocol for treatment of lymphoma. Treatment was associated with a rapid reduction of ferritin (see right figure, green line) and LDH (blue line) and return of fibrinogen (red line) to normal levels. During the 10 days of therapy for her hemophagocytosis-associated hematologic complications, she received 12 units of RBCs, 11 single-donor PLT transfusions, 5 units of FFP (blue arrows), and 6 units cryoprecipitate (fuchsia arrows). Between Days 10 and 19, she required an additional 8 units of RBCs, 13 units of single-donor PLTs, and 8 doses of granulocytes for chemotherapy-related pancytopenia and an associated fungal infection. Her cytopenias resolved by Day 20, and she was discharged in stable condition.Hemophagocytosis can occur in autoimmune diseases, viral infections, and T-cell lymphomas associated with hypercytokinemia and activation of the mononuclear phagocytic system. In addition to cellular engulfment, leading to anemia and thrombocytopenia, a coagulopathy attributed to hypofibrinogenemia can occur secondary to engulfment of fibrinogen via phagocytosis-associated membrane attack complex receptors on mononuclear phagocytes. Treatment is directed toward control of the underlying disease. Intensive blood bank support may be required during treatment of this syndrome.