T-Cell Prolymphocytic Leukemia Accompanied by Plural M-Proteins with Myelodysplastic Syndroxme in a Nonagenarian

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.

Hemolysis of Some Elderly MDS Cases Might be Related to Prolonged Parvovirus B19 Infection

“…Intracytoplasmic-iron demonstrated the viability of macrophage in heme recycling system [16] and can induce immunological abnormality in the host, which can lead to infection [17] as the macrophages and erythroid cells making physical contact can exert influences on each other [18]. Degraded immunoglobulin and/or auto-antibodies in serum has been observed in some MDS patients [10,19]. Immunoglobulin was found to be diminished in five of our six cases, indicating the occurrence of immunological abnormality.…”

“…The remaining three other cases showed abnormalities of soluble-IL2 receptor, ß2microglobulin, immunoglobulin or Coombs-test. From our previous reports [10,19], immunological abnormalities such as positive ANA were not the result of past infection by B19V but might be the hallmarks of compromised host such as MDS. Each of our cases showed clinical symptoms of immunological abnormality at the time of admission.…”

“…This is because their blood cells of the three lineages showed morphological abnormalities and their macrophage cytoplasm contains ingot irons, in addition to the general abnormal picture of their peripheral blood examination. Frequent presence of mast cells in BM blood smear also supported the diagnosis of MDS [10]. Generally, MDS in elderly person tends to manifest refractory cytopenia and hypo-hematopoiesis of erythrocytic precursor cells in bone marrow [1].…”