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An association between hypoplastic myelodysplastic syndrome and T-prolymphocytic leukaemia.

Arathi CA, Puttaraj KR, Shobha SN - J Lab Physicians (2011)

Bottom Line: 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.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Sree Siddhartha Medical College, Tumkur, Karnataka, India.

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.

No MeSH data available.


Related in: MedlinePlus

(a) Dyserythropoiesis with multinucleation in BMA. (b) Binucleated form. (c) Dysmegakaryopoiesis-cell block H and E, 40×. (d) dysmegakaryopoiesis BMA
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Figure 2: (a) Dyserythropoiesis with multinucleation in BMA. (b) Binucleated form. (c) Dysmegakaryopoiesis-cell block H and E, 40×. (d) dysmegakaryopoiesis BMA

Mentions: A 45-year-old male was admitted with generalized weakness, palpitation, gradual weight loss and frequent syncopal attacks for more than 2 years. On examination, the patient was afebrile, had severe pallor and mild splenomegaly was present. Previous reports showed progressive pancytopenia and hypocellular marrow since 2 years. The iron profile was: serum iron – 115.50 μg/dL, total iron binding capacity – 324.20 μg/dL, %transferrin saturation – 35.63%, ferritin – 177.10 ng/mL and markedly elevated B12 levels (>2000 pg/mL). Hemoglobin was 3.9 gm%, total leukocyte count was 2100 cells/cumm, platelet count was 70,000 cells/cumm and reticulocyte count was 0.8% at the time of admission. Pancytopenia [Figure 1a] was persistent on every visit and the peripheral smear showed normocytic and macrocytic RBCs, leukopenia, hypogranular and pseudo Pelger-Huet neutrophils [Figure 1b and 1c], which were characteristic with relative lymphocytosis and no blasts in the peripheral smear; the platelet count was reduced. The bone marrow aspiration smears and cell block preparation showed severe hypocellularity (<20% for his age) [Figure 1d]. Sparse erythroid cells with dyserythropoiesis [Figure 2a and 2b], minimal dysmyelopoietic (hypogranular and hyposegmented myeloid cells - 10%) and dysmegakaryopoietic (hypolobated and micromegakaryocytes [Figure 2c and 2d] features were evident with blasts <5% and absence of fibrosis in biopsy sections. Cytogenetic analysis could not yield any result due to inadequate cellularity at this stage. CD34 immunostaining revealed sparse precursors. The possibility of aplastic anemia and hypoplastic acute myeloid leukaemia (h-AML) were ruled out. Hams Test and HIV-I and II were negative. A diagnosis of primary H-MDS was made in the absence of chemotherapy and radiotherapy. The patient was transfusion dependent and later started on Thalidomide. On follow-up within few months, because of persistent pancytopenia, the patient was subjected for repeat bone marrow study, where the smear and section showed cellular marrow with diffuse infiltrate of mature lymphocytes (60%) [Figure 3a and b] with no evidence of improved hematopoiesis. The karyotype showed no abnormalities. The neoplastic cells showed a mature T-cell immunophenotype, CD3+, CD4+, CD5+, CD7+ and CD8+, and Tdt-, CD20-, CD23-, CD79a-, CD34-, Bcl2- and CyclinD1-, and was diagnosed as T-PLL. The patient was deteriorating and succumbed to death within a month of diagnosis.


An association between hypoplastic myelodysplastic syndrome and T-prolymphocytic leukaemia.

Arathi CA, Puttaraj KR, Shobha SN - J Lab Physicians (2011)

(a) Dyserythropoiesis with multinucleation in BMA. (b) Binucleated form. (c) Dysmegakaryopoiesis-cell block H and E, 40×. (d) dysmegakaryopoiesis BMA
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3118059&req=5

Figure 2: (a) Dyserythropoiesis with multinucleation in BMA. (b) Binucleated form. (c) Dysmegakaryopoiesis-cell block H and E, 40×. (d) dysmegakaryopoiesis BMA
Mentions: A 45-year-old male was admitted with generalized weakness, palpitation, gradual weight loss and frequent syncopal attacks for more than 2 years. On examination, the patient was afebrile, had severe pallor and mild splenomegaly was present. Previous reports showed progressive pancytopenia and hypocellular marrow since 2 years. The iron profile was: serum iron – 115.50 μg/dL, total iron binding capacity – 324.20 μg/dL, %transferrin saturation – 35.63%, ferritin – 177.10 ng/mL and markedly elevated B12 levels (>2000 pg/mL). Hemoglobin was 3.9 gm%, total leukocyte count was 2100 cells/cumm, platelet count was 70,000 cells/cumm and reticulocyte count was 0.8% at the time of admission. Pancytopenia [Figure 1a] was persistent on every visit and the peripheral smear showed normocytic and macrocytic RBCs, leukopenia, hypogranular and pseudo Pelger-Huet neutrophils [Figure 1b and 1c], which were characteristic with relative lymphocytosis and no blasts in the peripheral smear; the platelet count was reduced. The bone marrow aspiration smears and cell block preparation showed severe hypocellularity (<20% for his age) [Figure 1d]. Sparse erythroid cells with dyserythropoiesis [Figure 2a and 2b], minimal dysmyelopoietic (hypogranular and hyposegmented myeloid cells - 10%) and dysmegakaryopoietic (hypolobated and micromegakaryocytes [Figure 2c and 2d] features were evident with blasts <5% and absence of fibrosis in biopsy sections. Cytogenetic analysis could not yield any result due to inadequate cellularity at this stage. CD34 immunostaining revealed sparse precursors. The possibility of aplastic anemia and hypoplastic acute myeloid leukaemia (h-AML) were ruled out. Hams Test and HIV-I and II were negative. A diagnosis of primary H-MDS was made in the absence of chemotherapy and radiotherapy. The patient was transfusion dependent and later started on Thalidomide. On follow-up within few months, because of persistent pancytopenia, the patient was subjected for repeat bone marrow study, where the smear and section showed cellular marrow with diffuse infiltrate of mature lymphocytes (60%) [Figure 3a and b] with no evidence of improved hematopoiesis. The karyotype showed no abnormalities. The neoplastic cells showed a mature T-cell immunophenotype, CD3+, CD4+, CD5+, CD7+ and CD8+, and Tdt-, CD20-, CD23-, CD79a-, CD34-, Bcl2- and CyclinD1-, and was diagnosed as T-PLL. The patient was deteriorating and succumbed to death within a month of diagnosis.

Bottom Line: 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.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Sree Siddhartha Medical College, Tumkur, Karnataka, India.

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.

No MeSH data available.


Related in: MedlinePlus