Haemolytic Anaemia

The answer first

Haemolytic anaemia is a laboratory pattern — anaemia with a reticulocytosis, raised lactate dehydrogenase, raised unconjugated bilirubin, and reduced or absent haptoglobin — that the physician must classify by site (intra- or extra-vascular), mechanism (immune, mechanical, intrinsic red-cell, or external), and tempo (acute life-threatening or chronic compensated). ^[1] The answer-first move is to confirm haemolysis with a reticulocyte count and a blood film, then perform a direct antiglobulin (Coombs) test to split immune from non-immune causes, because that single test changes everything: a positive direct antiglobulin test sends the patient down an autoimmune pathway (warm autoimmune haemolytic anaemia, cold agglutinin disease, Evans syndrome, drug-induced immune haemolytic anaemia) for which the treatment is immunomodulation, whereas a negative test sends the patient towards a membrane, enzyme, haemoglobin, or microangiopathic cause for which the treatment is completely different. ^{[1] [13]}

For the FRACP candidate the haemolytic long case rewards a one-sentence synthesis ("A 68-year-old woman presenting with fatigue, jaundice, splenomegaly, a haemoglobin of 68 g/L with spherocytes on the film, and a positive IgG direct antiglobulin test — warm autoimmune haemolytic anaemia"), a problem list ordered by threat (transfusion-incompatibility, thrombosis, underlying lymphoproliferative disease), and a sequenced plan from immediate transfusion strategy through to definitive immunotherapy and secondary cause workup. ^[1]

References13ShowHide

1. [1]Jäger U, Barcellini W, Broome CM, et al. Diagnosis and treatment of autoimmune hemolytic anemia in adults: Recommendations from the First International Consensus Meeting Blood Rev, 2020.PMID 31839434
2. [2]Michel M, Terriou L, Roudot-Thoraval F, et al. A randomized and double-blind controlled trial evaluating the safety and efficacy of rituximab for warm auto-immune hemolytic anemia in adults (the RAIHA study) Am J Hematol, 2017.PMID 27696475
3. [3]Röth A, Barcellini W, D'Sa S, et al. Sutimlimab in Cold Agglutinin Disease N Engl J Med, 2021.PMID 33826820
4. [4]Berentsen S How I treat cold agglutinin disease Blood, 2021.PMID 33512410
5. [5]Bolton-Maggs PH, Langer JC, Iolascon A, Tittensor P, King MJ Guidelines for the diagnosis and management of hereditary spherocytosis--2011 update Br J Haematol, 2012.PMID 22055020
6. [6]Youngster I, Arcavi L, Schechmaster R, et al. Medications and glucose-6-phosphate dehydrogenase deficiency: an evidence-based review Drug Saf, 2010.PMID 20701405
7. [7]Uthman OA, Graves PM, Saunders R, et al. Safety of primaquine given to people with G6PD deficiency: systematic review of prospective studies Malar J, 2017.PMID 28830424
8. [8]Brandow AM, Liem RI Advances in the diagnosis and treatment of sickle cell disease J Hematol Oncol, 2022.PMID 35241123
9. [9]Vichinsky E, Hoppe CC, Ataga KI, et al. A Phase 3 Randomized Trial of Voxelotor in Sickle Cell Disease N Engl J Med, 2019.PMID 31199090
10. [10]Ataga KI, Kutlar A, Kanter J, et al. Crizanlizumab for the Prevention of Pain Crises in Sickle Cell Disease N Engl J Med, 2017.PMID 27959701
11. [11]Riley C, Kraft WK, Miller R Hydroxyurea in the sickle cell disease modern era Expert Rev Clin Pharmacol, 2024.PMID 39135533
12. [12]Scully M, Cataland SR, Peyvandi F, et al. Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura N Engl J Med, 2019.PMID 30625070
13. [13]Fattizzo B, Marchetti M, Michel M, et al. Diagnosis and management of Evans syndrome in adults: first consensus recommendations Lancet Haematol, 2024.PMID 38968944