Hemolytic crisis

Hemolytic crisis refers to an emergency situation in which there is a concomitant drop in hemoglobin in addition to accelerated hemolysis (Hoffbrand 2003).

Hemolytic crisis always represents an acute emergency situation (Herold 2022).

Nonfatal acute hemolytic transfusion reaction occurs in approximately 1: 6,000 - 80. 000 transfused units, and fatal in 1: 250,000 - 600. 000 transfused units (Link 2006).

Hemolytic crisis may occur acutely in the setting of, for example, a transfusion incident or may be caused by exacerbation of chronic hemolysis (Herold 2022).

In patients with glucose 6 -phosphate dehydrogenase deficiency , chloroquine can cause a hemolytic crisis (Dellas 2022).

Hemolytic crisis may result from oxidative stress (Hollstein 2023).

In a hemolytic crisis, the following symptoms may occur:

- usually dramatic onset with considerable pallor (Muntau 2018)

- Icterus with hyperbilirubinemia; typical of hemolysis is a markedly elevated indirect bilirubin and a normotensive direct bilirubin

- rapid Hb drop

- collapse

- Hemoglobinuria with beer-brown urine

- Acute renal failure in the setting of hemoglobinuria (Herold 2022)

- chills

- Fever

- Pain in the back, abdomen, head (Berger 2010)

The following laboratory valuesincrease during a hemolytic crisis:

- Free hemoglobin

- LDH

- Reticulocytes

- Indirect bilirubin (Rogobeanu 2021)

The following laboratory values drop:

- Haptoglobin (Rogobeanu 2021)

Coombs test

- Direct Coombs test strongly positive

- Indirect Coombs test only facultative positive (Muntau 2018)

Urine Findings:

- Urobilinogenuria

- Hemoglobinuria (Rogobeanu 2021).

In extravascular hemolysis, haptoglobin and free Hb in serum are usually normal, hemoglobinuria and hemosiderinuria do not occur. The only exception is hemolytic crisis in extravascular hemolysis. In this case, there is a decreased haptoglobin and hemoglobinuria (Herold 2023).

- Biliary tract disease, also associated with jaundice and fever (Herold 2022).

- Acute renal failure

- Cardiac decompensation (Muntau 2018)

- Allergic shock possible in a transfusion incident (Muntau 2018).

If hemoglobinuria exists in the setting of a hemolytic crisis, should be administered immediately:

- Corticosteroids Dosage recommendation: Decortin 50 - 100 mg.

Furthermore, it is necessary:

- Volume substitution with sodium bicarbonate to alkalinize the urine.

- Exchange transfusion or dialysis may be required (Rogobeanu 2021)

- Anticoagulation with Marcumar, but NO heparin because of possible complement activation (Wolff Weihrauch 2012).

Severe courses are possible, but spontaneous remission occurs in many cases (Muntau 2018).

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