ABSTRACT
Adult T-cell leukemia/lymphoma is an aggressive T-cell malignancy caused by the long-term infection of human T-cell lymphotropic virus type 1 (HTLV-1). Our understanding of clinical features still largely relies on the Shimoyama classification developed 30 years ago, which described the 4 clinical subtypes (the smoldering, chronic, lymphoma, and acute types) based on the manifestations of lymphocytosis, elevated lactate dehydrogenase, hypercalcemia, lymphadenopathy, and involvement of the skin, lung, liver, spleen, central nervous system, bone, ascites, pleural effusion, and gastrointestinal tract. HTLV-1-associated lymphoma has a variety of presentations but the presentation of massive lymphadenopathy and compression symptoms is rare and has not been emphasized in the literature. In this article, we describe 2 cases of adult T-cell leukemia/lymphomas that presented with massive cervical nodes or mediastinal nodes with compressing symptoms as the major presenting clinical features. Clinicians should remain aware of this type of presentation by HTLV-1-associated lymphoma, especially in patients who came from endemic areas, even if not all clinical features are present and particularly with hypercalcemia and lytic bone lesions.
Subject(s)
Human T-lymphotropic virus 1 , Leukemia-Lymphoma, Adult T-Cell , Lymphadenopathy , Lymphoma , Humans , Leukemia-Lymphoma, Adult T-Cell/complications , Leukemia-Lymphoma, Adult T-Cell/diagnosis , Lymphadenopathy/etiology , SkinABSTRACT
A 21-year-old man presented to the emergency department with generalised weakness, weight loss and decreased appetite for few weeks. He had evidence of severe pancytopenia and haemolysis. His peripheral smear with many schistocytes was suspicious for thrombotic thrombocytopenic purpura (TTP). He was supported with blood transfusions and daily plasmapheresis. His platelet counts worsened despite 4 days of therapy. Bone marrow biopsy was significant for hypercellular bone marrow with megaloblastic changes. Further workup revealed normal ADAMTS13 level, low vitamin B12, positive intrinsic factor antibodies and high methylmalonic acid. Diagnosis of pernicious anaemia was established and he was started on daily treatment with intramuscular vitamin B12 which subsequently improved his symptoms and haematological parameters. This report highlights the importance of checking vitamin B12 level in patients presenting with pancytopenia and TTP-like picture before making a diagnosis of TTP.
Subject(s)
Anemia, Pernicious/complications , Anemia, Pernicious/diagnosis , Pancytopenia/diagnosis , Pancytopenia/etiology , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/etiology , Anemia, Pernicious/therapy , Humans , Male , Pancytopenia/therapy , Purpura, Thrombotic Thrombocytopenic/therapy , Young AdultABSTRACT
We report a case of a 47-year-old male from West Africa who presented with sepsis and was found to have acute monoblastic leukemia associated with tetrasomy 8 detected on bone marrow samples. This was the only chromosomal abnormality found. Tetrasomy 8 is a rare genetic finding that has been reported in acute myeloid leukemia (AML), predominantly the monocytic lineage. It carries a poor prognosis with a high mortality rate.
