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CC BY-NC-ND 4.0 · J Lab Physicians 2011; 3(01): 049-051
DOI: 10.4103/0974-2727.78568
Case Report

An Association Between Hypoplastic Myelodysplastic Syndrome and T-Prolymphocytic Leukaemia

Arathi C A.
Department of Pathology, Sree Siddhartha Medical College, Tumkur, Karnataka, India
,
Puttaraj K R.
Department of Internal Medicine, Sree Siddhartha Medical College, Tumkur, Karnataka, India
,
Shobha S N.
Department of Pathology, Sree Siddhartha Medical College, Tumkur, Karnataka, India
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ABSTRACT

Myelodysplastic syndrome (MDS) represents one of the most challenging health-related problems in the elderly, characterized by dysplastic morphology in the bone marrow in association with ineffective hematopoiesis. Hypoplastic MDS (h-MDS) accounts for 12-17% of all patients with MDS and has yet to be shown to alter the disease course or prognosis. The concept that T-cell-mediated autoimmunity contributes to bone marrow failure in MDS has been widely accepted due to hematologic improvement after immunosuppressive therapy. T-cell expansion is known to occur in these patients, but development of chronic T-cell disorders, especially T-prolymphocytic leukemia (PLL) in a hypocellular MDS is extremely rare, which has an aggressive course. The possible explanation for the association between the two disorders is that T-PLL might arise from a clonally arranged MDS stem cell. We report a unique case of h-MDS with non-progressive pancytopenia and severe hypocellular marrow for 2 years, followed by T-PLL within few months.

Keywords

Chronic T-cell disorders - hypoplastic MDS - T-PLL


Publication History

Article published online:
29 January 2020

© 2011.

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