Successful donor-lymphocyte infusion for extreme immune-hemolysis following unrelated BMT in a patient with X-linked chronic granulomatous disease and McLeod phenotype

Kordes, Uwe; Binder, Thomas M.C.; Eiermann, Thomas; Hassenpflug-Diedrich, B; Hassan, Marwa A.; Beutel, Karin; Nagy, Marion; Kabisch, H; Schneppenheim, R.

doi:10.1038/bmt.2008.159

Cited by 13 publications

(7 citation statements)

References 9 publications

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“…He developed refractory severe hemolytic anemia after day+100 due to anti-Kx and anti-K and was found to have low donor T-cell chimerism, despite B-cell and erythroid conversion. His hemolytic anemia resolved after three doses of donor lymphocyte infusion [66].…”

Section: Non-abo Blood Groups Incompatibilitymentioning

confidence: 96%

Post–hematopoietic stem cell transplantation immune-mediated anemia: a literature review and novel therapeutics

et al. 2022

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Anemia following allogeneic hematopoietic stem cell transplantation (HCT) can be immune- or non-immune mediated. Auto- or alloimmunity due to blood groups incompatibility remain an important cause if post-HCT immune-mediated anemia. ABO incompatibility is commonly encountered in HCT and may lead to serious clinical complications including acute hemolysis, pure red cell aplasia, and passenger lymphocyte syndrome. It remains controversial whether ABO incompatibility may affect HCT outcomes, such as relapse, non-relapse mortality, graft-versus-host disease and survival. Non-ABO incompatibility is less frequently encounterd but can have similar complications to ABO incompatibility, causing adverse clinical outcomes. It is crucial to identify the driving etiology of post-HCT anemia in order to prevent and treat this condition. This requires a comprehensive understanding of the mechanism of anemia in blood group incompatible HCT, and the temporal association between HCT and anemia. In this review, we summarized the literature on post-HCT immune-mediated anemia with a focus on ABO and non-ABO blood group incompatibility, described the underlying mechanism of anemia, and outlined preventive and treatment approaches.

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Section: Non-abo Blood Groups Incompatibilitymentioning

confidence: 96%

Post–hematopoietic stem cell transplantation immune-mediated anemia: a literature review and novel therapeutics

et al. 2022

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“…The patient was transfusion naïve and did not have any known alloantibodies. To reduce the risk of allogeneic antibody formation peri-transplant, 1,2 CGD with MLS compatible red blood cell (RBC) units were unsuccessfully sought through the rare blood donor program, and a suitable directed donor was not identified. Therefore, the care team pursued the collection and freezing of autologous units.…”

Section: Cgd Chronic Granulomatous Disease Cryopreservation Mcleod Sy...mentioning

confidence: 99%

Cryopreservation of rare pediatric red blood cells for support following bone marrow transplant

et al. 2022

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Background: A 2-year-old, 10.8 kg male pediatric patient with X-linked chronic granulomatous disease (CGD) with McLeod syndrome (MLS) was scheduled for a hematopoietic stem cell transplant (HSCT). Identification of allogenic red blood cells (RBC) for post-transplant support was unsuccessful prompting the development of a customized method to collect and freeze rare autologous pediatric cells. Study Design and Methods: A protocol was developed for the collection of small volume pediatric whole blood (WB) via peripheral venipuncture with collection into 10 ml syringes containing anticoagulants. Additionally, a closed system RBC glycerolization and deglycerolization instrument was adapted to process small volume, non-leukoreduced WB. Both collection and WB processes were validated. In total 4 approximately 100 ml autologous units were collected and frozen. Two units were thawed, deglycerolized, and used for clinical transfusion support. To appreciate processing impacts on RBC rigidity, ektacytometry was performed on pre-processed and post-deglycerolization samples. Results: Free hemoglobin (HGB) of validation units after thawing/deglycerolization was <150 mg/dL with an average red cell recovery of 85%. These units also showed little difference between pre-and post-processing Lorrca deformability curves or membrane rigidity. Two pediatric units were thawed and deglycerolized for transfusion. Free HGB was 70 mg/dL and 50 mg/dL post-thaw, and these RBCs had a slight decrease in deformability and increased membrane rigidity.Discussion: Customized WB collection, glycerolization, freezing, and deglycerolization processes were developed to successfully support a pediatric patient with CGD and MLS after autologous HSCT. Both pediatric units Abbreviations: AABB, association for the advancement of blood and biotherapies; ACP-215, automated cell processor-215;

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“…The transfusion service was notified that compatible RBC units were not currently available and a suitable donor could not be identified. The risk of alloimmunization from non-MLS compatible units could complicate the post-transplant course (9)(10)(11), so an autologous whole blood donation program was developed by the transfusion service in conjunction with a local blood supplier to collect and freeze autologous units for a child of this size (~11 kg).…”

Section: Case Reportmentioning

confidence: 99%

Chronic granulomatous disease and McLeod syndrome: Stem cell transplant and transfusion support in a 2-year-old patient—a case report

et al. 2022

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Chronic granulomatous disease (CGD) with McLeod neuroacanthocytosis syndrome (MLS) is a contiguous gene deletion disorder characterized by defective phagocytic function and decreased Kell antigen expression. CGD cure is achieved through hematopoietic stem cell transplant (HSCT) usually in the peri-pubescent years. The presence of MLS makes peri-transfusion support complex, however. Herein, we present the youngest known case of HSCT for CGD in the setting of MLS. A 2-year-old male patient was diagnosed with CGD plus MLS. Due to the severity of the child’s systemic fungal infection at diagnosis, HSCT was deemed the best treatment option despite his small size and age. A related, matched donor was available, and a unique red blood cell support plan had been implemented. Reduced-intensity conditioning was used to reduce the transplant-related mortality risk associated with myeloablative protocols. The transplant course was uneventful; autologous red blood cell (RBC) transfusion support was successful and allowed for the avoidance of possible antibody formation if allogeneic units had been used. The patient achieved 1-year relapse-free survival. The developed protocols provide a viable path to transplant in the very young, and early transplant to cure could reduce disease-related morbidity.

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Successful donor-lymphocyte infusion for extreme immune-hemolysis following unrelated BMT in a patient with X-linked chronic granulomatous disease and McLeod phenotype

Cited by 13 publications

References 9 publications

Post–hematopoietic stem cell transplantation immune-mediated anemia: a literature review and novel therapeutics

Post–hematopoietic stem cell transplantation immune-mediated anemia: a literature review and novel therapeutics

Cryopreservation of rare pediatric red blood cells for support following bone marrow transplant

Chronic granulomatous disease and McLeod syndrome: Stem cell transplant and transfusion support in a 2-year-old patient—a case report

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