open access

Vol 52, No 4 (2021)
Review article
Published online: 2021-08-31
Submitted: 2021-07-07
Accepted: 2021-07-14
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Autoimmune cytopenias complicating hematopoietic cell transplantation

Jan Styczyński1, Katarzyna Gągola2, Agata Marjańska1
DOI: 10.5603/AHP.2021.0050
·
Acta Haematol Pol 2021;52(4):257-262.
Affiliations
  1. Department of Pediatric Hematology and Oncology, Jurasz University Hospital, Collegium Medicum, Nicolaus Copernicus University in Toruń, Bydgoszcz, Poland
  2. Regional Blood Transfusion Center (RCKiK), Bydgoszcz, Poland

open access

Vol 52, No 4 (2021)
REVIEW ARTICLE
Published online: 2021-08-31
Submitted: 2021-07-07
Accepted: 2021-07-14

Abstract

Immune cytopenias after allogeneic hematopoietic cell transplantation are rare, albeit increasingly recognized, complications. Autoimmune diseases are serious complications of HCT and include immune-mediated cytopenias i.e. autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP) and autoimmune neutropenia. Severe cytopenia is usually defined by decreases of hemoglobin concentration below 7 g/dL, of platelet count below 20 G/L, or of absolute granulocyte count below 0.5 G/L, and it is mediated by the presence of auto-antibodies. ITP occurring in combination with AIHA is known as Evans syndrome. Immune dysregulation is caused by impaired immune reconstitution and/or loss of self-tolerance. Primary risk factors of autoimmune cytopenias include: peripheral blood or cord blood as a stem cell source, unrelated HCT, non-malignant disease, use of alemtuzumab, acute/chronic graft-versus-host disease (GvHD), cytomegalovirus reactivation, infections, and, in pediatric settings, conditioning omitting total body irradiation. Diagnosis of autoimmune cytopenia is challenging due to a broad differential diagnosis: primary or secondary graft failure, infections, GvHD, disease relapse, drug-induced side effects, transplant-associated thrombotic microangiopathy, ABO-incompatibility, or disseminated intravascular coagulation. Treatment should be tailored to the individual patient, and ranges from watchful waiting to aggressive management in life-threatening situations. Apart from specific treatment adjusted for specific cytopenia, supportive care should include transfusions of leukocyte-reduced and irradiated red blood cell concentrates or pathogen-reduced platelet concentrates; treatment of infections and GvHD; modification of immunosuppression; and supplementation with microelements. Autoimmune cytopenias are usually highly resistant to standard therapy and are associated with increased risks of high morbidity and mortality, particularly when coexisting with other post-transplant complications.

Abstract

Immune cytopenias after allogeneic hematopoietic cell transplantation are rare, albeit increasingly recognized, complications. Autoimmune diseases are serious complications of HCT and include immune-mediated cytopenias i.e. autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP) and autoimmune neutropenia. Severe cytopenia is usually defined by decreases of hemoglobin concentration below 7 g/dL, of platelet count below 20 G/L, or of absolute granulocyte count below 0.5 G/L, and it is mediated by the presence of auto-antibodies. ITP occurring in combination with AIHA is known as Evans syndrome. Immune dysregulation is caused by impaired immune reconstitution and/or loss of self-tolerance. Primary risk factors of autoimmune cytopenias include: peripheral blood or cord blood as a stem cell source, unrelated HCT, non-malignant disease, use of alemtuzumab, acute/chronic graft-versus-host disease (GvHD), cytomegalovirus reactivation, infections, and, in pediatric settings, conditioning omitting total body irradiation. Diagnosis of autoimmune cytopenia is challenging due to a broad differential diagnosis: primary or secondary graft failure, infections, GvHD, disease relapse, drug-induced side effects, transplant-associated thrombotic microangiopathy, ABO-incompatibility, or disseminated intravascular coagulation. Treatment should be tailored to the individual patient, and ranges from watchful waiting to aggressive management in life-threatening situations. Apart from specific treatment adjusted for specific cytopenia, supportive care should include transfusions of leukocyte-reduced and irradiated red blood cell concentrates or pathogen-reduced platelet concentrates; treatment of infections and GvHD; modification of immunosuppression; and supplementation with microelements. Autoimmune cytopenias are usually highly resistant to standard therapy and are associated with increased risks of high morbidity and mortality, particularly when coexisting with other post-transplant complications.

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Keywords

autoimmune hemolytic anemia, AIHA, immune thrombocytopenia, ITP, autoimmune neutropenia, AIN

About this article
Title

Autoimmune cytopenias complicating hematopoietic cell transplantation

Journal

Acta Haematologica Polonica

Issue

Vol 52, No 4 (2021)

Article type

Review article

Pages

257-262

Published online

2021-08-31

DOI

10.5603/AHP.2021.0050

Bibliographic record

Acta Haematol Pol 2021;52(4):257-262.

Keywords

autoimmune hemolytic anemia
AIHA
immune thrombocytopenia
ITP
autoimmune neutropenia
AIN

Authors

Jan Styczyński
Katarzyna Gągola
Agata Marjańska

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