A 54-year-old male patient was admitted to the hospital for a 3-month history of epigastric pain and vomiting, weight loss of approximately 10 kg and progressive dyspnea.
There were regular general performance status (Eastern Cooperative Oncology Group) conditions of 3 and significant dyspnea.
Physical examination revealed an erythematous rash in the anterior region of the chest, with nodular lesions in the arms and a large increase in tumor volume in the right forearm, 8 cm in diameter.
Multiple cervical, axillary and inguinal bilateral adenopathies up to 2 cm. In addition, signs of right pleural effusion, liver 15 cm below the costal margin (BRC) and spleen 7 cm
A scanner showed an enlarged lymph node occupying pulmonary media and palatine tonsils, suggesting involvement of Waldayer's ring, adenopathies in the submandibular space of 3.3 x 3.1 cm.
Hepatomegaly (not measured), moderate ascites and poorly defined retroperitoneal and periportal lymphadenopathy.
Laboratory tests showed Hb 10.1 g/dL, leukocytes 633.700 x mm3, with 98% of lymphocytes and platelets of 37.000 x mm3, normal calcium and renal function, serum albumin of 2.4 g/L.
Peripheral blood smear: 90% mature small lymphocytes pleo; basophilic cytoplasm and irregular nucleus, some brainoids.
Pleural effusion puncture: lymphocytes with the same characteristics as peripheral blood.
Myelogram showed 90% of similar lymphocytes.
The immunophenotype by flow cytometry reported 95% of mature T lymphoid lineage cells, intense CD45, CD2 (+), CD3 (+), CD5 (+), CD7 (+), CD25 (+) expression.
CD34, HLA-DR and TdT negative markers.
Cytogenetic analysis showed complex chromosomal abnormalities with structural alterations in chromosomes X, 5, 8, 11, 13, 18 and monosomy 6, 10, 11, 12 and 13.
Viral serology for HBV/HCV/HIV/HTLV1 was negative.
The diagnosis of cerebriform cell variant T-cell prolymphocytic leukemia was made. Chemotherapy was initiated CHOP (hallucinamide, doxorubicin, vincristine and prednisone).
This achieved a transient disappearance of skin lesions, pleural effusion and leukocytosis, which reappeared on day 8 of chemotherapy.
It was decided to start rescue chemotherapy ESHAP (etoposide, cytarabine, cisplatin, methylprednisolone), without clinical response.
The patient died two months after diagnosis due to progressive disease.
