Purpura of the Face and Neck: An Atypical Clinical Presentation Revealing a Hepatosplenic T Cell Lymphoma
A 62-year-old woman presented to our department with an asymptomatic cutaneous eruption of the face extending to the neck that had appeared 2 weeks earlier. She did not report any infectious symptoms in the past weeks. Fever, night sweats or weight loss were denied. There was neither anamnestical atopy nor known allergies. Medication was limited to estradiol (substitution therapy) and occasional ibuprofen intake. Physical examination revealed non-palpable petechial purpura distributed on the face and extending to the neck (fig. ). Peripheral white blood cell count, repartition and hemoglobin concentration were in the normal range, while platelet count was decreased to 90 G/l. Coagulation tests (aPTT, prothrombin time, fibrinogen) showed no anomaly. Lactate dehydrogenase, aspartate aminotransferase, and alanine aminotransferase were slightly elevated (LDH 316 U/l, ASAT 58 U/l, ALAT 52 U/l); alkaline phosphatase, gamma-glutamyl transferase, total bilirubin and serum creatinine concentrations were all within normal limits. Protein electrophoresis and immune subtraction allowed ruling out a monoclonal gammopathy. Erythrocyte sedimentation rate and serum complement concentrations were within normal limits; antinuclear antibodies, ANCAs (c-ANCA, p-ANCA and x-ANCA) as well as serologies for HIV, HBV, HCV, CMV, EBV and PVB19 were all negative. Histopathological examination of the purpuric macules showed extravasation of erythrocytes and a slight lymphohistiocytic perivascular inflammation in the superficial and mid dermis, without evidence of vasculitis (fig. ). Amplification of the TCR in the skin biopsy showed no monoclonality. Direct immunofluorescence was negative. Given the particular distribution of the purpura in the upper part of the body, a cervico-thoracoabdominal CT scan was performed, which allowed to rule out a compressive component on the venous flow. It showed a multinodular goiter as well as a slight splenomegaly (12 cm). At that time, the patient was released with a diagnosis of idiopathic thrombocytopenic purpura.\nTwo months later, the patient complained of night sweats and the purpuric eruption had extended to the whole body (fig. ). Physical examination revealed a hepatosplenomegaly without palpable adenopathy. Thrombocytopenia had progressed to 30 G/l, LDH was 921 U/l, ALAT 179 U/l, ASAT 236 U/l, alkaline phosphatase 156 U/l, gamma-glutamyl transferase 96 U/l and total bilirubin was 31 μmol/l. Other laboratory examinations were within normal limits. A thoracoabdominal CT scan showed progression of the splenomegaly (from 12 to 16 cm). The examination of the bone marrow biopsy and aspirate showed an atypical lymphoid infiltrate composed of medium-sized cells with oval to irregular nuclei, moderately abundant cytoplasms, featuring a predominantly sinusoidal distribution. Immunophenotyping (immunohistochemistry and flow cytometry) revealed atypical lymphoid cells which were CD2+/CD3+/CD4–/CD5–/CD7–/CD8–/CD30– and focal expression of the T cell-restricted intracellular antigen-1 (TIA1). T cells strongly expressed the gamma/delta TCR and were negative for TCR beta F1. A monoclonal rearrangement of the TCR-gamma gene was demonstrated by PCR. We retained a diagnosis of HSTL and CHOP chemotherapy was administered together with a prophylactic intrathecal mixed chemotherapy (cytarabine, methotrexate, hydrocortisone). After two courses of treatment, the patient reported a diminution of night sweats, although the thoracoabdominal CT scan showed no modification, in particular no change in hepato- and splenomegaly; clinically, the purpuric rash showed a dramatic regression paralleled by a normalization of the platelet count (platelet count 157 G/l). After six courses of chemotherapy, as the infiltration of the bone marrow by neoplastic T cells persisted, the patient underwent a well-tolerated autologous bone marrow graft. Two months later, no recurrence of the purpura was observed. A close clinical, biological and radiological follow-up for early detection of a potential HSTL recurrence is currently ongoing at the oncological unit.