AbstractPDF
Abstract
Primary cutaneous gamma-delta T-cell lymphoma
(PCGD-TCL) is rare and only represents 1% of all
cutaneous T-cell lymphomas. To our knowledge, only
40 cases have been described. It often presents with
generalised skin lesions, preferentially affecting the
extremities. There is a well-documented association
with haemophagocytic syndrome. Treatment is difficult
since PCGD-TCL is often resistant to chemotherapy and
radiotherapy. Most case reports describe an aggressive
clinical course with an estimated mean survival of 15
months. We present a 72-year-old female patient with stage IV primary cutaneous gamma-delta T-cell lymphoma. Our patient presented with fever, night sweats and multiple skin lesions (figure 1). Computed axial tomography of chest and abdomen revealed multiple solid nodular lesions in both kidneys. During admission a subconjunctival lesion appeared and progressed rapidly (figure 2). Histopathological examination of skin biopsy revealed infiltration of atypical lymphocytes with hyperchromatic irregular nuclei. Immunophenotyping pattern of skin biopsy was compatible with PCGD-TLC. Clonal gamma-delta T-cells were also detected by immunohistochemical analysis of peripheral blood and bone marrow. Polymerase chain reaction amplification revealed clonal rearrangement of the T-cell receptor gamma chain gene. These findings together were consistent with stage IV primary cutaneous gamma-delta T-cell lymphoma. The rapid progression of the subconjunctival extra-nodal manifestation is characteristic for the aggressive course of this lymphoma. Our patient was treated with two cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone). However, her clinical condition deteriorated rapidly. She declined further therapy and died within three months of initial presentation.
