Case report
A 50-year-old female with no significant past medical history presented with hectic fever, weight loss, asthenia and abdominal pain 3 weeks before admission in our department. Closer examination revealed multiple telangiectasias located on the chest and upper back, with proximo-distal extension (Fig.1). Physical examination displayed a voluminous hepato-splenomegaly. The biological analysis showed a hemophagocytic syndrome (HS) with inflammatory syndrome (CRP 41 mg/L). A small population of Double negative abnormal T population CD2+ CD3+ CD4- CD8- CD5-CD56- CD16+ CD17+ accounting for 27.9% of total lymphocytes, presenting γδTCR was identified using flow cytometry. Medullar karyotype identified 44,X,-X,+8,add(10)(q26),-11,-21[6]/46,XX[14]. Plasma Vascular Endothelial Growth Factor (VEGF) level was significantly elevated (1140 pg/mL, normal value inferior to 500 pg/mL). A mutation ofSTAT5B T628S was identified by molecular biology. Computed tomography (CT) scan showed voluminous hepatosplenomegaly. Positron emission tomography-CT revealed diffuse hypermetabolism in the hepatosplenomegaly and regarding the osteomedullary area. The course was marked by a worsening of the abdominal pain revealing a subcapsular intraparenchymal spleen rupture. The splenectomy associated with liver biopsy concluded Hepatosplenic T CD4-/CD8- lymphoma stage IVBb (Fig 2.). Remarkably, the patient’s global state improved after splenectomy. After 2 lines of chemotherapy, she received allogeneic stem cell transplantation from an unrelated donor.
Here we present the case of an hepato-splenic-Tγδ-cell lymphoma interestingly occurring in a non-immunocompromised patient, with profuse telangiectasias giving originally misleading orientation towards the diagnosis of B angiotropic lymphoma. We observed an increased blood VEGF level that can be possibly correlate with clinical telangiectasias. Gamma-delta-hepato-splenic T-cell lymphoma is a rare entity of primary extranodal disease and represent less than 5% of all PTCL cases. The prognosis of this lymphoma is poor requiring multiple lines of chemotherapy and allogeneic stem cell transplantation.