Non Hodgkin T cell lymphoma: an atypical clinical\npresentation
We describe a 46-year-old, black male patient, born in Cape Verde. He first noted,\nmonths prior to presentation, a dermatosis that almost exclusively involved his face and\ntrunk consisting of multiple small monomorphic papules with an erythematous smooth\nsurface. The patient reported dysesthesia at the distal ends of both feet and\nparoxistical difficulty in mobilizing both feet. These symptoms suggested the existence\nof a sensory motor polyneuropathy.A cutaneous biopsy was performed motivated by these\nfindings. The histopathological evaluation revealed perivascular and periadnexal\ninflammatory infiltration of nerve bundles with lymphocytic permeation which, with\nsimultaneous findings of cutaneous involvement and sensory motor polyneuropathy, could\nbe consistent with the diagnosis of leprosy. The patient was therefore referred for a\nconsultation at the hospital regarding the possibility of Hansen's disease. The physical\nexamination revealed a dermatosis involving the face and the upper torso that consisted\nof multiple infracentimetric monomorphic papules and plaques with a smooth erythematous\nsurface ( and ). There was no hypoesthesia in relation to skin lesions.\nExamination of ganglionic chains revealed several adenopathies below the chin and in\naxillary chains. The adenopathies that were found were over one centimeter in diameter,\nwith a hard consistency. Palpation of the abdomen revealed no masses or organomegaly.\nThe neurological examination did not reveal thickening or pain upon palpation of the\nnerve roots. There was slight hypoesthesia at the distal end of both feet, with\ndiminished thermal, tactile and pain sensitivity.\nThe patient also reported weight loss of 12 kg(>10%) during the past year which had\nnever been taken into consideration. The patient denied any other constitutional\nsymptoms, including fever, anorexia and malaise.\nThe supplementary study revealed: normocytic normochromic anemia(10.4 g/dL), white blood\ncell count with lymphocytosis(3500) and thrombocytopenia (94000),VS 71mm in 1st hour,\nβ2-microglobulin(8.0 mg/dL) and LDH(689 U/L) and negative results for HIV, HTLV, EBV.\nPCR applied to a biopsy performed from the synovial tissue proved negative for\nM. leprae.\nBody axial tomography showed severe ganglion involvement of the axillary and inguinal\nlymph nodes and mild splenomegaly with multiple small parenchymal nodules. The skin\nbiopsy revealed diffuse lymphoid infiltration in the dermis composed of CD3, CD4\npositive T-cells, some of which had CD8 positivity (, and ). The axillary lymph node biopsy identified a lymphocytic\nproliferation composed of small cells with CD2, CD3 positive cells and CD7, CD8, CD20\nnegative cells, and TIA expression allowing the diagnosis of T-cell lymphoma with\ncytotoxic expression (). The study of\nT-cell receptors rearrangement by PCR confirmed the monoclonality of the cell population\nbased on the analysis of gamma genes.The absence of typical clinical lesions and the\ntypical histological markers of Hansen's disease, the successively negative nasal smears\nand skin swabs, together with the PCR detection technique for M. leprae,\nallowed for the exclusion of a Hansen's disease diagnosis. The evaluation by a\nneurologist allowed a correlation between lymphoma and demyelinating neuropathy to be\ncharacterized as a rare paraneoplastic syndrome.\nThe patient was started on chemotherapy with CHOP (cyclophosphamide, doxorubicin,\nvincristine and prednisolone), which led to partial remission of the cutaneous lesions\n() but with subsequent loss of\nresponse. Second line therapeutic management with ESHAP (etoposide, methylprednisolone,\ncytarabine and cisplatin ) was then initiated with no noticeable clinical response. The\npatient was then started on a CMOPP chemotherapy regimen (cyclophosphamide,\nmechlorethamine, vincristine, procarbazine and prednisolone), but at 12 months follow-up\nthere was invariable progression of the systemic disease, resulting in the patient´s\ndeath.