Autoimmune hemolytic anemia: transfusion challenges and solutions

Barros, Melca Maria Oliveira; Langhi, Dante Mário; Bordin, José Orlando

doi:10.2147/ijctm.s81870

Cited by 10 publications

(8 citation statements)

References 27 publications

(119 reference statements)

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“…31 Transfusions to maintain Hb at a clinically acceptable level are indicated when a child develops hypoxaemia, cardiac decompensation or a rapidly progressive anaemia. 6 Children tend to compensate better for a falling Hb and transfusions are usually given when Hb is <5-6 g/L (above this consider child's clinical status). 29 The laboratory must be informed of the diagnosis so that pretransfusion testing can be performed above the thermal amplitude of the D-L antibody to avoid agglutination and delays to transfusions.…”

Section: How Does a Positive D-l Test And Subsequent Diagnosis Of Pch Influence Management Of A Child Who Presented With An Acute Haemolymentioning

confidence: 99%

How to use Donath-Landsteiner test to diagnose paroxysmal cold haemoglobinuria (PCH)

Williams

Jayaprakash

Kithany

et al. 2021

Arch Dis Child Educ Pract Ed

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Paroxysmal cold haemoglobinuria (PCH) accounts for around a third of cases of autoimmune haemolytic anaemia in children. PCH is caused by an autoantibody that fixes complement to red cells at low temperatures and dissociates at warmer temperatures (a biphasic haemolysin), triggering complement-mediated intravascular haemolysis. Named the Donath-Landsteiner (D-L) antibody after its discoverers, it is usually formed in response to infection and demonstrates specificity for the ubiquitous red cell P-antigen. A D-L test can be used to detect the presence of the D-L autoantibody in the patients’ serum. Here we discuss the use of the D-L test in identifying PCH in a 2-year-old boy who presented with haemolytic anaemia. A summary of the key information can be found in the infographic.

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Section: How Does a Positive D-l Test And Subsequent Diagnosis Of Pch Influence Management Of A Child Who Presented With An Acute Haemolymentioning

confidence: 99%

How to use Donath-Landsteiner test to diagnose paroxysmal cold haemoglobinuria (PCH)

Williams

Jayaprakash

Kithany

et al. 2021

Arch Dis Child Educ Pract Ed

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“…23,24 There are currently no data regarding the treatment of acute AIHA with anti-En a specificity. Although it is recognized that warm AIHA has particular transfusion challenges, 25 specificity of the autoantibody might not matter as no patient or RBC survival benefit was demonstrated. 26,27 However, since fatal warm AIHA with anti-En a specificity was previously reported, rapid recognition and initiation of rituximab therapy may be lifesaving.…”

Section: Discussionmentioning

confidence: 99%

Acute autoimmune hemolytic anemia due to anti‐En^a autoantibody successfully treated with rituximab

et al. 2017

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BACKGROUND Autoimmune hemolytic anemia (AIHA) due to anti‐Ena has been previously reported in association with massive intravascular hemolysis, disseminated intravascular coagulation, and fatal outcomes. Here we report a case of successfully treated AIHA due to anti‐Ena. CASE REPORT A 69‐year‐old male with a past medical history of cirrhosis due to nonalcoholic steatohepatitis status post–orthotopic liver transplant presented with 1‐month history of progressive anemia. At presentation, his hemoglobin (Hb) was 5.6 g/dL, hematocrit (Hct) 16%, reticulocytes 0.3%, direct bilirubin (bili) 4 g/dL, lactate dehydrogenase 533 units/L (reference, 125‐220 units/L), and haptoglobin 254 mg/dL (reference, 40‐273 mg/dL). Blood bank testing revealed an autoantibody present in his plasma and a direct antiglobulin test positive for immunoglobulin G (IgC) but negative for complement. He received 1 unit of an incompatible blood group O phenotypically matched red blood cell unit. RESULTS Over the course of the next 5 days, the Hb and Hct decreased to 4.1 g/dL and 12%, respectively, direct bili increased to 12.3 mg/day, reticulocytes slightly increased to 0.9%, and haptoglobin decreased to less than 8 mg/dL. Marrow study showed a hypercellular marrow with erythroid hyperplasia. Additional workup performed at a reference laboratory identified an anti‐Ena autoantibody. He received prednisone and weekly rituximab infusions and was monitored weekly. At the 2‐month visit, Hb and Hct were 10 g/dL and 32%, respectively. CONCLUSION Unlike two of the previously reported fatal cases of AIHA with anti‐Ena specificity, this 69‐year‐old male treated with weekly rituximab infusion underwent clinical recovery and significant anemia improvement.

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“…A serological work-up to investigate the presence of RBC alloantibodies is needed. 17 Several tests are available to detect alloantibodies in patients with warm autoantibodies. The most effective is the adsorption test that removes autoantibody from the patient's serum and allows for detection and identification of alloantibodies in the adsorbed serum.…”

Section: Transfusing Aiha Patientsmentioning

confidence: 99%

“…12,15 Adequate testing for alloantibodies may take several hours (usually 4-6 h) to be completed, so in urgent cases and seriously ill patient, physicians must balance the risk of withholding transfusion and the possible benefit of correcting Hb level. 15,17 Washing of RBCs is done for a variety of reasons, such as to remove excess electrolyte, allergens, and cytokines and or to prevent the effects of storage lesion. The washing process will remove 95%-99% of RBC supernatant, which contains plasma proteins, electrolytes, WBCs, platelets, cellular debris, etc.…”

Section: Transfusing Aiha Patientsmentioning

confidence: 99%

“…Blood transfusion in AIHA patients with severe anemia is needed to maintain their Hb at a clinically acceptable level. 17 There has been a lack of evidence transfusion guidelines in AIHA patients; therefore, no specific Hb threshold can be recommended. Physicians should not feel obligated to transfuse based on the Hb value specified by any guideline.…”

Section: Transfusing Aiha Patientsmentioning

confidence: 99%

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Red Blood Cell Transfusion for Pediatric Autoimmune Hemolytic Anemia in an Emergency Situation

Andriastuti¹,

Ritchie²,

Kosasih³

et al. 2022

ejki

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Autoimmune hemolytic anemia (AIHA) is often presented with severe anemia. Currently, several centers in Indonesia use washed red blood cells (RBCs) to transfuse AIHA patients. However, washed RBCs are not widely available, causing a delay in therapy. We herein report two cases of pediatric AIHA who presented with severe anemia with hemoglobin level of 4.1 g/dL and 5.3 g/dL. Both patients were delayed for two and five days to be given blood transfusion because of the unavailability of washed RBC in Sorong General Hospital and Fatmawati Hospital and must wait to be referred to RSCM to receive washed RBCs. We conducted literature searches to review several articles that focus on AIHA transfusion management. AIHA is characterized by the production of autoantibodies directed against antigens on the surface of RBCs. RBC washing is not currently required in AIHA patients. It is also not widely available, takes a long time to process, and can cause a delay in emergency cases. Although RBC transfusion is not contraindicated in AIHA, its use should be limited to life-threatening cases of anemia with signs of cardiac and neurological compromises. In emergency cases, ABOmatched and Rhesus-matched RBCs can be safely administered if alloantibodies are reasonably excluded based on the previous transfusion and pregnancy history. ABO-matched and Rh-matched RBC transfusion is safe as AIHA’s transfusion management for patients with severe anemia with clinical symptoms.

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Autoimmune hemolytic anemia: transfusion challenges and solutions

Cited by 10 publications

References 27 publications

How to use Donath-Landsteiner test to diagnose paroxysmal cold haemoglobinuria (PCH)

How to use Donath-Landsteiner test to diagnose paroxysmal cold haemoglobinuria (PCH)

Acute autoimmune hemolytic anemia due to anti‐En^a autoantibody successfully treated with rituximab

Red Blood Cell Transfusion for Pediatric Autoimmune Hemolytic Anemia in an Emergency Situation

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Autoimmune hemolytic anemia: transfusion challenges and solutions

Cited by 10 publications

References 27 publications

How to use Donath-Landsteiner test to diagnose paroxysmal cold haemoglobinuria (PCH)

How to use Donath-Landsteiner test to diagnose paroxysmal cold haemoglobinuria (PCH)

Acute autoimmune hemolytic anemia due to anti‐Ena autoantibody successfully treated with rituximab

Red Blood Cell Transfusion for Pediatric Autoimmune Hemolytic Anemia in an Emergency Situation

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Acute autoimmune hemolytic anemia due to anti‐En^a autoantibody successfully treated with rituximab