Microangiopathic hemolytic anemia: Difference between revisions
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== | ==Background== | ||
*Abbreviation: MAHA | |||
*A variety of conditions that result in damaged endothelium of small vessels causing platelet aggregation and fibrin deposition which leads to shearing forces within these small vessels causing an intravascular fragmentation hemolysis | |||
==Clinical Features== | |||
*Clinical features + heme labs with red blood cell fragments or schistocytes.<ref name="guidelines">Scully. Marie et al. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012 Aug;158(3):323-35. [http://www.bcshguidelines.com/documents/BJH_TTP_Guideline_0512.pdf PDF]</ref> | |||
*May have [[hemorrhage|bleeding]] and [[thromboembolism|thrombosis]] | |||
*Signs and symptoms of [[anemia]] (fatigue, pallor, palpitations, etc.) | |||
*[[Jaundice]] and scleral icterus | |||
*Signs/symptoms of underlying disease process | |||
==Diagnosis== | ==Differential Diagnosis== | ||
{{Hemolytic anemia DDX}} | |||
==Evaluation== | |||
== | |||
*Peripheral smear, hemoglobin, platelets, PT/INR, PTT, fibrinogen, FDPs, D-dimer, haptoglobin, LDH, bilirubin | *Peripheral smear, hemoglobin, platelets, PT/INR, PTT, fibrinogen, FDPs, D-dimer, haptoglobin, LDH, bilirubin | ||
**Schistocytes (aka Helmet cells) secondary to fragmentation hemolysis classic finding on peripheral smear | **Schistocytes (aka Helmet cells) secondary to fragmentation hemolysis classic finding on peripheral smear | ||
===Lab Findings=== | |||
*Schistocytes | |||
*High: +/- LDH and unconjugated bilirubin | |||
*Low: Hgb, platelets, +/- haptoglobin | |||
===General Rules=== | |||
* | *[[DIC]]= '''elevated''' PT/INR/PTT/FDPs/D-dimer, '''low''' fibrinogen<ref name="guidelines"></ref> | ||
* | *[[TTP]]/[[HUS]] = '''normal''' PT/INR/PTT/FDPs/D-dimer/fibrinogen<ref name="guidelines"></ref> | ||
==Management== | |||
==Disposition== | |||
==See Also== | ==See Also== | ||
*[[ | *[[Anemia]] | ||
==References== | |||
<references/> | |||
[[Category:Heme/Onc]] | [[Category:Heme/Onc]] | ||
Latest revision as of 04:51, 1 October 2019
Background
- Abbreviation: MAHA
- A variety of conditions that result in damaged endothelium of small vessels causing platelet aggregation and fibrin deposition which leads to shearing forces within these small vessels causing an intravascular fragmentation hemolysis
Clinical Features
- Clinical features + heme labs with red blood cell fragments or schistocytes.[1]
- May have bleeding and thrombosis
- Signs and symptoms of anemia (fatigue, pallor, palpitations, etc.)
- Jaundice and scleral icterus
- Signs/symptoms of underlying disease process
Differential Diagnosis
Microangiopathic Hemolytic Anemia (MAHA)
- Disseminated Intravascular Coagulation (DIC)
- Thrombotic Thrombocytopenic Purpura (TTP)
- Hemolytic Uremic Syndrome (HUS)
- HELLP syndrome
- Heparin-Induced Thrombocytopenia (HIT)
- Hereditary spherocytosis
- Paroxysmal nocturnal hemoglobinuria (PNH)
- Malignant hypertension
- Scleroderma
- Antiphospholipid Syndrome (APS)
- Other medical causes: malignancy, renal allograft rejection, vasculitides like polyarteritis nodosa and Wegener's granulomatosis
- Drugs: chemotherapy; Clopidogrel (Plavix) associated with TTP
- Nonvascular causes: prosthetic valve (more common with mechanical, more common at aortic valve), LVAD, TIPS, coil embolization, patched AV shunt, AVM
Evaluation
- Peripheral smear, hemoglobin, platelets, PT/INR, PTT, fibrinogen, FDPs, D-dimer, haptoglobin, LDH, bilirubin
- Schistocytes (aka Helmet cells) secondary to fragmentation hemolysis classic finding on peripheral smear
Lab Findings
- Schistocytes
- High: +/- LDH and unconjugated bilirubin
- Low: Hgb, platelets, +/- haptoglobin