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.