Abstract
T-cell Pro-Lymphocytic Leukemia (T-PLL) is a rare disease caused by malignancy of mature post-thymic T-cell. Myelodysplastic Syndrome (MDS) is caused by abnormal differentiation of myeloid lineage cells resulting in myeloid leukemia. Both of these hematological disorders are frequently diagnosed in elderly persons. Myeloid lineage is believed to be situated at the position far from lymphoid one as it is regarded on a tree diagram about the blood cell maturation. We reported herein a rare case of T-PLL accompanied by plural M-proteins with MDS a nonagenarian. The 96 years old patient was admitted to our hospital because of lymphocytosis and abnormal lymphocytes. From the bone marrow aspirate, biopsy and hematological findings, abnormalities were observed in cells of three different lineages, namely, (i) neutrophils with hypersegmented-nuclei (ii) erythroblasts with nuclear division, large platelets and megakaryoblastic cells, and (iii) reticulum cells with phagocytosed iron in their cytoplasm. Lymphocytes showed CD3 (+), CD4 (±), CD5 (+), CD8 (+), CD10 (+), CD56 (-) and CD57 (-). Mast cells and large lymphocytic cells with nuclear pockets were also seen. Anti-HTLV-1 antibody was negative. Soluble IL-2 receptor was significantly elevated to 7910 U/ml. Both IgGλ and IgGκM-protein were detected. No Bence-Jones protein was detected in the urine. Chromosomal abnormality of 45 (X, 0) or loss of Y chromosome was also demonstrated. Due to his high age, it was difficult to classify his condition according to the conventional classification. Thus, what we had experienced is truly a rare case that coincidentally showed T-PLL and MDS with plural M-proteins.
Highlights
T-cell prolymphocytic leukemia (T-PLL), which is commonly accompanied by splenomegaly, is a mature post-thymic T-cell malignancy with an aggressive clinical course
This disease was previously known as T-cell chronic lymphocytic leukemia but has been reclassified as T-PLL based on the post-thymic T-cell markers [2]
Myelodysplastic syndrome (MDS) is a hematological disease caused by abnormal differentiation of myeloid lineage cells in which some patients will develop to several forms of myeloid leukemia [4]
Summary
T-cell prolymphocytic leukemia (T-PLL), which is commonly accompanied by splenomegaly, is a mature post-thymic T-cell malignancy with an aggressive clinical course It is an extremely rare disorder accounting for the lowest number of all the lymphocytic leukemia [1]. Patient Y.S, a 96-yr-old Japanese male, was referred from a practicing physician to our Nemuro city hospital because of a WBC count of 26.6 × 109/L with 75% lymphocytes He was admitted to our hospital for a detailed examination of bone marrow aspiration and biopsy to make a definitive diagnosis of lymphocytic leukemia. He did not show any abnormal physical findings such as skin lesion nor lymphadenopathy on admission, except for splenomegaly. They were (i) the neutrophils, most of which have hypersegmented nuclei and with few cytoplasmic granules, (ii) the erythroblasts with nuclear division, the large platelets and the abnormal megakaryoblastic cells, and (iii) the reticulum cells which a e b f
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