Autoimmune hemolytic anemia differential diagnosis: Difference between revisions

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|Microangiopathic hemolytic anemia  
|Microangiopathic hemolytic anemia  
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* Abnormal immune activation<ref name="pmid25705656">{{cite journal| author=Berentsen S, Sundic T| title=Red blood cell destruction in autoimmune hemolytic anemia: role of complement and potential new targets for therapy. | journal=Biomed Res Int | year= 2015 | volume= 2015 | issue=  | pages= 363278 | pmid=25705656 | doi=10.1155/2015/363278 | pmc=4326213 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25705656  }} </ref>
* [[Thrombotic thrombocytopenia purpura]]
* Failure of self vs. non-self recognition<ref name="pmid25705656">{{cite journal| author=Berentsen S, Sundic T| title=Red blood cell destruction in autoimmune hemolytic anemia: role of complement and potential new targets for therapy. | journal=Biomed Res Int | year= 2015 | volume= 2015 | issue=  | pages= 363278 | pmid=25705656 | doi=10.1155/2015/363278 | pmc=4326213 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25705656  }} </ref>
* [[Hemolytic uremic syndrome]]
* [[Infections]]
* [[Disseminated intravascular coagulation]]
* [[Chronic lymphocytic leukemia]]
* [[Non-Hodgkin's lymphoma]]
* [[Rheumatologic disease]]
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* Polyclonal antibody production that binds to and targets [[red blood cells]] for destruction intravascurly or extravascularly<ref name="pmid25705656">{{cite journal| author=Berentsen S, Sundic T| title=Red blood cell destruction in autoimmune hemolytic anemia: role of complement and potential new targets for therapy. | journal=Biomed Res Int | year= 2015 | volume= 2015 | issue=  | pages= 363278 | pmid=25705656 | doi=10.1155/2015/363278 | pmc=4326213 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25705656  }} </ref>
* Formation of small vessel microthombi
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* [[Schistocytes]] on peripheral blood smear
* [[Schistocytes]] on peripheral blood smear
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* Treatment of the underlying cause
* Treatment of the underlying cause
* Plasmapheresis if [[thrombotic thrombocytopenia purpura]] (TTP) is the underlying cause
* Plasmapheresis if [[thrombotic thrombocytopenia purpura]] is the underlying cause
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* Can be life-threatening pending the underlying cause
* Can be life-threatening pending the underlying cause

Revision as of 18:58, 12 April 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Assosciate Editor(s)-In-Chief: Prashanth Saddala M.B.B.S; Shyam Patel [2]

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Overview

Differentiating Autoimmune hemolytic anemia from other Diseases

Diagnosis is made by first ruling out other causes of hemolytic anemia, such as G6PD, thalassemia, sickle-cell disease, etc.


Characteristic Causes Pathophysiology Laboratory abnormalities Physical examination Therapy Other associations
Autoimmune hemolytic anemia
  • Polyclonal antibody production that binds to and targets red blood cells for destruction intravascurly or extravascularly[1]
  • Removal of offending agent
  • Corticosteroids
  • Cyclophosphamide
  • Cyclosporine A
  • Azathioprine
  • Rituximab
  • Splenectomy
  • Hemolysis can occur at warm or cold temperatures
Microangiopathic hemolytic anemia
  • Formation of small vessel microthombi
  • Bleeding
  • Thrombosis
  • Pyrexia
  • Altered mental status
  • Neurologic deficits
  • Impaired urine output
  • Can be life-threatening pending the underlying cause
  • TTP required immediate treatment
Paroxysmal cold hemoglobinuria
  • Biphasic hemolysin (IgG) that binds red blood cells and low temperatures and triggers complement-mediated intravascular hemolysis at warm temperatures[2]
  • Associated with syphilis[2]
  • Maternal IgG can cross the placenta and affect the fetus[2]
Paroxysmal nocturnal hemoglobinuria
  • Genetic defect in anchoring proteins for complement factors on red blood cells
  • Hemolysis due to loss of complement inhibition on the red blood cell surface, which in turn is due to defect in CD55 (decay accelerating factor) and CD59 (membrane inhibitor of reactive lysis) or other glycophosphatidylinositol-anchored proteins on the red blood cell membrane
  • Absence of CD55 and CD59 by flow cytometry
  • Splenomegaly
  • Abdominal tenderness
  • Pallor
Hereditary spherocytosis[3]
  • Mutation in ankyrin[3]
  • Mutation in alpha- or beta-spectrin[3]
  • Mutation in band 3[3]
  • Mutation in protein 4.2[3]
  • Positive eosin-5-maleimide binding to red blood cells[3]
  • Positive osmotic fragility testing[3]
  • Spherocytes on peripheral blood smear
  • Can be autosomal dominant or recessive
Pernicious anemia[4]
  • Autoimmune gastritis[4]
  • Production of anti-intrinsic factor antibodies[4]
  • Production of anti-parietal cell antibodies[4]
  • Impaired vitamin B12 absorption due to absence of intrinsic factor
  • Low vitamin B12 level
  • Presence of anti-intrinsic factor antibodies
  • Presence of anti-parietal cell antibodies
  • Associated with diabetes, thyroid disease, vitiligo and other autoimmune conditions
Chronic lymphocytic leukemia[5]
  • Mutations in hematopoietic stem cells and B lymphocytes
  • Clonal proliferation of malignant B lymphocytes
  • Elevated absolute lymphocyte count
  • Anemia (Rai stage III) and thrombocytopenia (Rai stage IV)
  • Chemotherapy with rituximab
  • Ibrutinib
  • Venetoclax
  • Secondary autoimmune hemolytic anemia occurs in 10-25% of patients with CLL
  • Treatment with corticosteroids or anti-leukemic therapy will correct the underlying anemia

References

  1. 1.0 1.1 1.2 Berentsen S, Sundic T (2015). "Red blood cell destruction in autoimmune hemolytic anemia: role of complement and potential new targets for therapy". Biomed Res Int. 2015: 363278. doi:10.1155/2015/363278. PMC 4326213. PMID 25705656.
  2. 2.0 2.1 2.2 2.3 Akpoguma AO, Carlisle TL, Lentz SR (2015). "Case report: paroxysmal cold hemoglobinuria presenting during pregnancy". BMC Hematol. 15: 3. doi:10.1186/s12878-015-0023-7. PMC 4334594. PMID 25699184.
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Gallagher PG (2013). "Abnormalities of the erythrocyte membrane". Pediatr Clin North Am. 60 (6): 1349–62. doi:10.1016/j.pcl.2013.09.001. PMC 4155395. PMID 24237975.
  4. 4.0 4.1 4.2 4.3 4.4 Chan CQ, Low LL, Lee KH (2016). "Oral Vitamin B12 Replacement for the Treatment of Pernicious Anemia". Front Med (Lausanne). 3: 38. doi:10.3389/fmed.2016.00038. PMC 4993789. PMID 27602354.
  5. Kipps TJ, Stevenson FK, Wu CJ, Croce CM, Packham G, Wierda WG; et al. (2017). "Chronic lymphocytic leukaemia". Nat Rev Dis Primers. 3: 16096. doi:10.1038/nrdp.2016.96. PMC 5336551. PMID 28102226.

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