Multiorgan failure in a fatal case of autoimmune hemolytic anemia

Anandappa, Annabelle J.; Stefely, Jonathan A.; Dzik, Walter H.; Waheed, Anem

doi:10.1111/trf.16513

Cited by 3 publications

(1 citation statement)

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“…Many AIHA patients solely require outpatient care. However, several case reports and series have illustrated that AIHA can culminate into a fulminant and even fatal clinical course ( 7 – 10 ). These patients may deteriorate rapidly due to massive hemolysis, leading to organ failure, and require repeated blood transfusions and treatment in an intensive care unit (ICU).…”

Section: Introductionmentioning

confidence: 99%

Severe autoimmune hemolytic anemia; epidemiology, clinical management, outcomes and knowledge gaps

Mulder,

Evers,

de Haas

et al. 2023

Front. Immunol.

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Autoimmune hemolytic anemia (AIHA) is an acquired hemolytic disorder, mediated by auto-antibodies, and has a variable clinical course ranging from fully compensated low grade hemolysis to severe life-threatening cases. The rarity, heterogeneity and incomplete understanding of severe AIHA complicate the recognition and management of severe cases. In this review, we describe how severe AIHA can be defined and what is currently known of the severity and outcome of AIHA. There are no validated predictors for severe clinical course, but certain risk factors for poor outcomes (hospitalisation, transfusion need and mortality) can aid in recognizing severe cases. Some serological subtypes of AIHA (warm AIHA with complement positive DAT, mixed, atypical) are associated with lower hemoglobin levels, higher transfusion need and mortality. Currently, there is no evidence-based therapeutic approach for severe AIHA. We provide a general approach for the management of severe AIHA patients, incorporating monitoring, supportive measures and therapeutic options based on expert opinion. In cases where steroids fail, there is a lack of rapidly effective therapeutic options. In this era, numerous novel therapies are emerging for AIHA, including novel complement inhibitors, such as sutimlimab. Their potential in severe AIHA is discussed. Future research efforts are needed to gain a clearer picture of severe AIHA and develop prediction models for severe disease course. It is crucial to incorporate not only clinical characteristics but also biomarkers that are associated with pathophysiological differences and severity, to enhance the accuracy of prediction models and facilitate the selection of the optimal therapeutic approach. Future clinical trials should prioritize the inclusion of severe AIHA patients, particularly in the quest for rapidly acting novel agents.

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Section: Introductionmentioning

confidence: 99%

Severe autoimmune hemolytic anemia; epidemiology, clinical management, outcomes and knowledge gaps

Mulder,

Evers,

de Haas

et al. 2023

Front. Immunol.

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Multiple drugs

2021

Reactions Weekly

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Multiple drugs Lack of efficacy: case reportA 65-year-old man exhibited a lack of efficacy after receiving treatment with eculizumab, etoposide, immune-globulin, methylprednisolone, and rituximab for autoimmune haemolytic anaemia [not all dosages stated].The man was hospitalised with a one week history of reddish-brown urine, dyspnoea on exertion, jaundice, and lightheadedness. His medical history included an unprovoked deep vein thrombosis that was treated with rivaroxaban. He denied having been exposed to blood products before, taking new drugs, or bleeding recently. Physical examination revealed hypotension, splenomegaly, marked pallor, and scleral icterus. Consequently, he underwent several laboratory investigations and was diagnosed with autoimmune haemolytic anaemia. Initially, he received treatment with IV immune-globulin [immunoglobulin]. Additionally, he also received concomitant therapy with unspecified glucocorticoids and blood transfusions. His anaemia worsened despite administering treatment. Later on, he received treatment with IV 600mg of eculizumab, a high dose of methylprednisolone, and 1g of IV rituximab. Despite these treatments, his haemolysis continued, as measured by increased LDH and the need for frequent transfusions. On Day 7, his hepatic function deteriorated, and he was diagnosed with deep venous thromboses in both lower limbs. He then underwent a splenectomy on day 8. He required transfusions despite the splenectomy, and he was managed with etoposide IV 50 mg/m2 on days 13 and 17. On Day 16, repeated testing of his plasma revealed continued high titer IgG autoantibodies, indicating that past treatment had only a minor suppressive impact. On day 15, he developed progressive liver failure. Finally, he developed treatment refractory autoimmune haemolytic anaemia, despite receiving medications [lack of efficacy of immune-globulin, rituximab, eculizumab and methylprednisolone, and etoposide]. Eventually, acute renal and hepatic failure, venous thrombosis, progressive encephalopathy, worsening haemodynamic instability and deteriorating coagulopathy exacerbated his course, and he died of multiorgan failure on day 18.

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