Pure red cell aplasia: Association with large granular lymphocyte leukemia and the prognostic value of cytogenetic abnormalities

Martha Lacy, Paul J. Kurtin, Ayalew Tefferi

Research output: Contribution to journalArticle

90 Citations (Scopus)

Abstract

From 1980 through 1994, we identified 47 adult patients with acquired pure red cell aplasia (median age, 64 years; range, 22 to 84 years). Associated clinical disorders included T-cell large granular lymphocytic (LGL) leukemia, thymoma, chronic lymphocytic leukemia, and non-Hodgkin's lymphoma. Review of bone marrow findings in 40 patients showed absence of erythroid precursors in 14 patients and rare pronormoblasts in 26. None had morphologic evidence of myelodysplasia. T-cell receptor gene rearrangement studies with Southern blot technique in 14 patients showed clonal rearrangements in nine. Karyotypic analyses performed in 28 patients showed clonal abnormalities in four. Overall, 28 of 47 patients (60%) responded to immunosuppressive therapy, but none were the patients with cytogenetic abnormalities. There was a trend toward superior response to immunosuppressive agents in the patients with T- cell LGL leukemia. Cyclophosphamide, with or without corticosteroids, was the most useful treatment agent. Cyclosporine A was effective for refractory disease. Neither the presence of an associated clinical disorder nor the existence of detectable erythroid precursors affected overall survival. We conclude that (1) T-cell LGL leukemia is the disorder most commonly associated with pure red cell aplasia, (2) the presence of clonal cytogenetic abnormality predicts poor response to immunosuppressive therapy, and (3) oral cyclophosphamide and cyclosporine A are effective treatment regimens.

Original languageEnglish (US)
Pages (from-to)3000-3006
Number of pages7
JournalBlood
Volume87
Issue number7
StatePublished - Apr 1 1996

Fingerprint

Large Granular Lymphocytic Leukemia
Pure Red-Cell Aplasia
T-cells
Lymphocytes
Immunosuppressive Agents
Chromosome Aberrations
Cells
Cyclophosphamide
Cyclosporine
T-Cell Antigen Receptor
Refractory materials
Adrenal Cortex Hormones
Bone
B-Cell Chronic Lymphocytic Leukemia
Genes
T-Lymphocyte Gene Rearrangement
T-Cell Receptor Genes
Erythroblasts
Thymoma
Therapeutics

ASJC Scopus subject areas

  • Hematology

Cite this

Pure red cell aplasia : Association with large granular lymphocyte leukemia and the prognostic value of cytogenetic abnormalities. / Lacy, Martha; Kurtin, Paul J.; Tefferi, Ayalew.

In: Blood, Vol. 87, No. 7, 01.04.1996, p. 3000-3006.

Research output: Contribution to journalArticle

@article{01d46e16216f4022abc55af21570de6b,
title = "Pure red cell aplasia: Association with large granular lymphocyte leukemia and the prognostic value of cytogenetic abnormalities",
abstract = "From 1980 through 1994, we identified 47 adult patients with acquired pure red cell aplasia (median age, 64 years; range, 22 to 84 years). Associated clinical disorders included T-cell large granular lymphocytic (LGL) leukemia, thymoma, chronic lymphocytic leukemia, and non-Hodgkin's lymphoma. Review of bone marrow findings in 40 patients showed absence of erythroid precursors in 14 patients and rare pronormoblasts in 26. None had morphologic evidence of myelodysplasia. T-cell receptor gene rearrangement studies with Southern blot technique in 14 patients showed clonal rearrangements in nine. Karyotypic analyses performed in 28 patients showed clonal abnormalities in four. Overall, 28 of 47 patients (60{\%}) responded to immunosuppressive therapy, but none were the patients with cytogenetic abnormalities. There was a trend toward superior response to immunosuppressive agents in the patients with T- cell LGL leukemia. Cyclophosphamide, with or without corticosteroids, was the most useful treatment agent. Cyclosporine A was effective for refractory disease. Neither the presence of an associated clinical disorder nor the existence of detectable erythroid precursors affected overall survival. We conclude that (1) T-cell LGL leukemia is the disorder most commonly associated with pure red cell aplasia, (2) the presence of clonal cytogenetic abnormality predicts poor response to immunosuppressive therapy, and (3) oral cyclophosphamide and cyclosporine A are effective treatment regimens.",
author = "Martha Lacy and Kurtin, {Paul J.} and Ayalew Tefferi",
year = "1996",
month = "4",
day = "1",
language = "English (US)",
volume = "87",
pages = "3000--3006",
journal = "Blood",
issn = "0006-4971",
publisher = "American Society of Hematology",
number = "7",

}

TY - JOUR

T1 - Pure red cell aplasia

T2 - Association with large granular lymphocyte leukemia and the prognostic value of cytogenetic abnormalities

AU - Lacy, Martha

AU - Kurtin, Paul J.

AU - Tefferi, Ayalew

PY - 1996/4/1

Y1 - 1996/4/1

N2 - From 1980 through 1994, we identified 47 adult patients with acquired pure red cell aplasia (median age, 64 years; range, 22 to 84 years). Associated clinical disorders included T-cell large granular lymphocytic (LGL) leukemia, thymoma, chronic lymphocytic leukemia, and non-Hodgkin's lymphoma. Review of bone marrow findings in 40 patients showed absence of erythroid precursors in 14 patients and rare pronormoblasts in 26. None had morphologic evidence of myelodysplasia. T-cell receptor gene rearrangement studies with Southern blot technique in 14 patients showed clonal rearrangements in nine. Karyotypic analyses performed in 28 patients showed clonal abnormalities in four. Overall, 28 of 47 patients (60%) responded to immunosuppressive therapy, but none were the patients with cytogenetic abnormalities. There was a trend toward superior response to immunosuppressive agents in the patients with T- cell LGL leukemia. Cyclophosphamide, with or without corticosteroids, was the most useful treatment agent. Cyclosporine A was effective for refractory disease. Neither the presence of an associated clinical disorder nor the existence of detectable erythroid precursors affected overall survival. We conclude that (1) T-cell LGL leukemia is the disorder most commonly associated with pure red cell aplasia, (2) the presence of clonal cytogenetic abnormality predicts poor response to immunosuppressive therapy, and (3) oral cyclophosphamide and cyclosporine A are effective treatment regimens.

AB - From 1980 through 1994, we identified 47 adult patients with acquired pure red cell aplasia (median age, 64 years; range, 22 to 84 years). Associated clinical disorders included T-cell large granular lymphocytic (LGL) leukemia, thymoma, chronic lymphocytic leukemia, and non-Hodgkin's lymphoma. Review of bone marrow findings in 40 patients showed absence of erythroid precursors in 14 patients and rare pronormoblasts in 26. None had morphologic evidence of myelodysplasia. T-cell receptor gene rearrangement studies with Southern blot technique in 14 patients showed clonal rearrangements in nine. Karyotypic analyses performed in 28 patients showed clonal abnormalities in four. Overall, 28 of 47 patients (60%) responded to immunosuppressive therapy, but none were the patients with cytogenetic abnormalities. There was a trend toward superior response to immunosuppressive agents in the patients with T- cell LGL leukemia. Cyclophosphamide, with or without corticosteroids, was the most useful treatment agent. Cyclosporine A was effective for refractory disease. Neither the presence of an associated clinical disorder nor the existence of detectable erythroid precursors affected overall survival. We conclude that (1) T-cell LGL leukemia is the disorder most commonly associated with pure red cell aplasia, (2) the presence of clonal cytogenetic abnormality predicts poor response to immunosuppressive therapy, and (3) oral cyclophosphamide and cyclosporine A are effective treatment regimens.

UR - http://www.scopus.com/inward/record.url?scp=0029897898&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0029897898&partnerID=8YFLogxK

M3 - Article

C2 - 8639922

AN - SCOPUS:0029897898

VL - 87

SP - 3000

EP - 3006

JO - Blood

JF - Blood

SN - 0006-4971

IS - 7

ER -