Hemolytic anemia: Difference between revisions

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==Differential Diagnosis==
==Differential Diagnosis==
===Acquired Hemolytic Anemia===
===Acquired Hemolytic Anemia===
====Microangiopathic Hemolytic Anemia====
====Microangiopathic Hemolytic Anemia====
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==Evaluation==
==Evaluation==
*Labs
===Workup===
** CBC
* CBC
***Look for low h/h
**Look for low h/h
***Low platelet count -> microangiopathic hemolytic anemia
**Low platelet count -> microangiopathic hemolytic anemia
** Blood smear is one of the most important tests to obtain
* Blood smear is one of the most important tests to obtain
** Reticulocyte Count
* Reticulocyte Count
** CMP
* CMP
*** Most important:  indirect bilirubin and creatinine
** Most important:  indirect bilirubin and creatinine
** UA, Urine Hemoglobin, Urine Hemosiderin, Upreg
* [[UA]], Urine Hemoglobin, Urine Hemosiderin, Upreg
** PT/INR
* PT/INR
** Hemolysis Labs
* Hemolysis Labs
*** LDH
** LDH
***Haptoglobin
**Haptoglobin
***Fibrinogen  
**Fibrinogen  
** Direct Anti-Globulin Test or Coombs test
* Direct Anti-Globulin Test or Coombs test
** If concern for Malaria:
* If concern for Malaria:
***Thick and Thin prep
**Thick and Thin prep
*** Parasitemia  
** Parasitemia  
**HIV
*HIV
**Blood cultures
*Blood cultures
**Urine Cultures
*Urine Cultures
**Consider LP if neuro symptoms
*Consider [[LP]] if neuro symptoms


==Lab Interpretation==
===Lab Interpretation===
{| {{table}}
{| {{table}}
| align="center" style="background:#f0f0f0;"|'''Microangiopathic Hemolytic Anemia'''
| align="center" style="background:#f0f0f0;"|'''Microangiopathic Hemolytic Anemia'''

Revision as of 05:54, 19 October 2016

General

  • Wide variety of clinical presentation given the large differential diagnosis of Hemolytic Anemia
  • Symptoms, Physical Exam, and Labs findings differ depending on intravascular vs. extravascular hemolysis, acute vs. chronic
  • Common theme: low hemoglobin and hematocrit, reticulocytosis, elevated indirect bilirubin
  • Most important lab to elucidate diagnosis is blood smear
  • Divided by etiology: Acquired and Hereditary
    • Acquired:
      • Microangiopathic, Autoimmune, infection
    • Hereditary:
      • Sickle Cell Disease, Thalassemia, G6PD, Hereditary Spherocytosis
  • Most common emergent presentations are due to acute intravascular hemolytic anemias

Clinical Features

History

  • Anemia symptoms
    • Dyspnea
    • Fatigue
    • DOE
  • Stigmata of intravascular hemolysis
    • Jaundice (new onset)
    • Dark urine
    • Changes in stool color
    • Neuro (TTP)
      • Headache
      • AMS
      • Seizure
      • Focal deficit
      • Coma
    • Rectal Bleeding (HUS)
  • Stigmata of thrombocytopenia
    • Petechiae
    • Bleeding
  • Stigmata of extravascular RBC destruction
    • Abdominal pain
    • Back pain
  • Systemic Symptoms
    • Fevers
    • Night sweats
    • Weight loss
    • Confusion
  • Other
    • Trauma
    • Initiation of new medication
    • Recent travel
    • Recent bug bites

PMH

  • Hereditary Spherocytosis
  • Sickle Cell Disease
  • G6PD Deficiency recently started new medication:
    • Dapsone
    • Phenazopyridine
    • Nitrofurantoin
    • Primaquine
    • Rasburicase
    • Methylene blue
    • Tolonium chloride (toluidine blue)
  • Malignancy
  • Renal Failure
  • Connective Tissue Disease
  • Family history of Anemia/Bleeding

Physical Exam

  • Cardiovascular
    • Tachycardia with flow murmur
    • Heart murmur – prosthetic heart valve
  • Abdominal Exam
    • Hepatomegaly
    • Splenomegaly
    • Ascites
  • Skin
    • Petechiae
    • Bruising
    • Lymphadenopathy
    • Brown recluse spider bites

Differential Diagnosis

Acquired Hemolytic Anemia

Microangiopathic Hemolytic Anemia

  • HUS
  • TTP
  • DIC
  • Malignant Hypertension

Autoimmune

  • Warm Antibody Autoimmune Hemolytic Anemia

Infection

  • Malaria
  • Babesia

Other

  • Brown recluse spider venom

Hereditary/Congenital Hemolytic Anemia

  • G6PD – medication induced hemolytic anemia
  • Sickle Cell Disease
  • Thalassemia
  • Hereditary Spherocytosis

Evaluation

Workup

  • CBC
    • Look for low h/h
    • Low platelet count -> microangiopathic hemolytic anemia
  • Blood smear is one of the most important tests to obtain
  • Reticulocyte Count
  • CMP
    • Most important: indirect bilirubin and creatinine
  • UA, Urine Hemoglobin, Urine Hemosiderin, Upreg
  • PT/INR
  • Hemolysis Labs
    • LDH
    • Haptoglobin
    • Fibrinogen
  • Direct Anti-Globulin Test or Coombs test
  • If concern for Malaria:
    • Thick and Thin prep
    • Parasitemia
  • HIV
  • Blood cultures
  • Urine Cultures
  • Consider LP if neuro symptoms

Lab Interpretation

Microangiopathic Hemolytic Anemia H/H Platelets Indirect Bili Creatinine Blood Smear PT/INR LDH Haptoglobin Fibrinogen DAT/Coombs Test Thick/Thin Prep
HUS low low elevated highly elevated schistocytes wnl elevated low wnl negative negative
TTP low low elevated elevated schistocytes wnl elevated low wnl negative negative
DIC low low elevated elevated schistocytes elevated/elevated elevated low low negative negative
Malignant Hypertension low low elevated variable schistocytes elevated low negative negative
Autoimmune H/H Platelets Indirect Bili Creatinine Blood Smear PT/INR LDH Haptoglobin Fibrinogen DAT/Coombs Test Thick/Thin Prep
Warm Antibody AHA low wnl elevated spherocytes elevated low wnl positive negative
Infection H/H Platelets Indirect Bili Creatinine Blood Smear PT/INR LDH Haptoglobin Fibrinogen DAT/Coombs Test Thick/Thin Prep
Malaria low wnl elevated see thick/thin prep variable elevated low wnl negative paracytes
Babesia see thick/thin prep negative paracytes
Other H/H Platelets Indirect Bili Creatinine Blood Smear PT/INR LDH Haptoglobin Fibrinogen DAT/Coombs Test Thick/Thin Prep
Brown rec low wnl elevated elevated positive negative

Management

  • ABC’s and Resuscitation if necessary
  • 2 Large bore IVs
  • Emergent hematology consultation if patient is very ill appearing

Acquired Hemolytic Anemia

Microangiopathic Hemolytic Anemia

  • HUS
    • Supportive Care
      • Hydration
      • Pain control
    • Hemodialysis if acute renal failure
    • Do NOT give Antibiotics: results in increased expression of Shiga Toxin from E. Coli O157:H7
  • TTP
    • Avoid platelet transfusion, except in life-threatening bleeding or intracranial hemorrhage
    • Plasma Exchange
    • If Plasma exchange cannot be performed immediately, give FFP and pheresis later.
    • Infusion with factor VIII concentrate
  • DIC
    • Platelets if count is <50,000 and/or significant bleeding
    • pRBC if active bleeding or hemodynamically unstable
    • FFP if active bleeding
    • Cryoprecipitate if fibrinogen <150 and bleeding
    • TXA is only indicated for active or massive bleeding
  • Malignant hypertension
    • Decreased blood pressure as clinically indicated

Autoimmune

  • Warm Antibody Autoimmune Hemolytic Anemia
    • High-dose corticosteroids PO (1-2mg/kg per day for 3-4 weeks)
    • Monoclonal antibodies and immunosuppressive agents
    • Plasma exchange for severe hemolysis
    • Allogeneic RBC transfusion for life-threatening anemia

Infection

  • Babesia
    • Mild/moderate Disease
      • Atovaquone 750mg PO q12hr plus Azithromycin 500mg PO on day 1, then 250mg/day PO
    • Severe Disease
      • Adult: Clindamycin 300-600mg IV qid or 600mg PO TID Plus Quinine 650mg PO TID x 7-10 days
      • Consider exchange transfusion if parasitemia >10%
    • Supportive treatment

Other

  • Brown recluse spider venom
    • Supportive care
      • Hemodynamic support with fluids and pressers if necessary
      • Blood product transfusion if necessary

Hereditary/Congenital Hemolytic Anemia

  • G6PD
    • Stop new medications
    • Treat any infections aggressively
    • Ovoid oxidant drugs
    • Blood transfusion if severe illness
  • Sickle Cell Disease
    • Hemolytic anemia in SCD is typically chronic in nature not requiring treatment in the ED.
  • Thalassemia
    • Hemolytic anemia in Thalassemia is typically chronic in nature not requiring treatment in the ED.
  • Hereditary Spherocytosis
    • Hemolytic anemia in Hereditary Spherocytosis is typically chronic in nature not requiring treatment in the ED.

Disposition

Admit

Also See

External Links

References

  • Dhaliwal G, Cornett P, Tierney LM Jr. Hemolytic Anemia. Am Fam Physician. 2004 Jun 1;69(11):2599-606.
  • Rother RP, Bell L, Hillmen P, Gladwin MT. The Clinical Sequelae of Intravascular Hemolysis and Extracellular Plasma HemoglobinA Novel Mechanism of Human Disease. JAMA. 2005;293(13):1653-1662. doi:10.1001/jama.293.13.1653
  • Barcellini W, Fattizzo B. Clinical applications of hemolytic markers in the differential diagnosis and management of hemolytic anemia. Disease markers. 2015 Dec 27;2015.
  • Bain BJ. Diagnosis from the blood smear. New England Journal of Medicine. 2005 Aug 4;353(5):498-507.
  • Tefferi A. Anemia in adults: a contemporary approach to diagnosis. In Mayo Clinic Proceedings 2003 Oct 31 (Vol. 78, No. 10, pp. 1274-1280). Elsevier.
  • Wada H, Matsumoto T, Yamashita Y. Diagnosis and treatment of disseminated intravascular coagulation (DIC) according to four DIC guidelines. Journal of Intensive Care. 2014;2(1):15. doi:10.1186/2052-0492-2-15.
  • McDade, Jenny et al. Brown Recluse Spider (Loxosceles reclusa) Envenomation Leading to Acute Hemolytic Anemia in Six Adolescents. The Journal of Pediatrics, Volume 156, Issue 1, 155 - 157
  • Albert E. Anderson, Paul B. Cassaday, George R. Healy. Babesiosis in Man: Sixth Documented Case American Journal of Clinical Pathology Nov 1974, 62 (5) 612 618; DOI:10.1093/ajcp/62.5.612