Hemolytic anemia: Difference between revisions

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==General==
==Background==
*Wide variety of clinical presentation given the large differential diagnosis of Hemolytic Anemia
*Wide variety of clinical presentation given the large differential diagnosis  
*Symptoms, Physical Exam, and Labs findings differ depending on intravascular vs. extravascular hemolysis, acute vs. chronic
*Clinical presentation and lab findings differ depending on intravascular vs. extravascular hemolysis, acute vs. chronic
*Common theme: low hemoglobin and hematocrit, reticulocytosis, elevated indirect bilirubin
*Common findings
**low hemoglobin and hematocrit
**reticulocytosis
**elevated indirect bilirubin
*Most important lab to elucidate diagnosis is blood smear
*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
*Most common emergent presentations are due to acute intravascular hemolytic anemias
===Etiologies===
''Divided by etiology: acquired vs. hereditary''
*Acquired:
**Microangiopathic ([[MAHA]]), [[Autoimmune hemolytic anemia|autoimmune]], infection
*Hereditary:
**[[Sickle cell disease]], [[thalassemia]], [[G6PD]], [[hereditary spherocytosis]]


==Clinical Features==
==Clinical Features==
===History===
===History===
*Anemia symptoms
*[[Anemia]] symptoms
**Dyspnea
**[[Dyspnea]]/dypsnea on exertion
**Fatigue
**Fatigue
**DOE
*Stigmata of intravascular hemolysis
*Stigmata of intravascular hemolysis
**Jaundice (new onset)
**[[Jaundice]] (new onset)
**Dark urine
**Dark urine
**Changes in stool color
**Changes in stool color
** Neuro (TTP)
**Neuro ([[TTP]])
*** Headache
***[[Headache]]
*** AMS
***[[Altered mental status]]
*** Seizure
***[[Seizure]]
*** Focal deficit
***[[Focal neuro deficits]]
*** Coma
***[[Coma]]
** Rectal Bleeding (HUS)
**[[Rectal bleeding]] ([[HUS]])
*Stigmata of thrombocytopenia
*Stigmata of [[thrombocytopenia]]
**Petechiae
**[[Petechiae]]
**Bleeding
**Bleeding
*Stigmata of extravascular RBC destruction
*Stigmata of extravascular RBC destruction
**Abdominal pain
**[[Abdominal pain]]
**Back pain
**[[Back pain]]
*Systemic Symptoms
*Systemic Symptoms
**Fevers
**[[Fever]]
**Night sweats
**Night sweats
**Weight loss
**Weight loss
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**Initiation of new medication
**Initiation of new medication
**Recent travel
**Recent travel
**Recent bug bites
**Recent [[insect bites]]


===PMH===
===PMH===
*Hereditary Spherocytosis
*[[Hereditary spherocytosis]]
*Sickle Cell Disease
*[[Sickle cell disease]]
*G6PD Deficiency recently started new medication:
*[[G6PD]] Deficiency recently started new medication:
** Dapsone
**[[Dapsone]]
**Phenazopyridine
**Phenazopyridine
**Nitrofurantoin
**[[Nitrofurantoin]]
**Primaquine
**Primaquine
**Rasburicase
**[[Rasburicase]]
**Methylene blue
**[[Methylene blue]]
**Tolonium chloride (toluidine blue)
**Tolonium chloride (toluidine blue)
*Malignancy
*Malignancy
*Renal Failure
*[[Renal failure]]
*Connective Tissue Disease
*[[Connective tissue disease]]
* Family history of Anemia/Bleeding
*Family history of Anemia/Bleeding


===Physical Exam===
===Physical Exam===
*Cardiovascular
*Cardiovascular
**Tachycardia with flow murmur
**[[Tachycardia]] with flow murmur
** Heart murmur – prosthetic heart valve
**Heart murmur – prosthetic heart valve
*Abdominal Exam
*Abdominal Exam
** Hepatomegaly
**Hepatomegaly, splenomegaly
** Splenomegaly
**[[Ascites]]
** Ascites
*Skin
*Skin
**Petechiae
**[[Petechiae]]
**Bruising
**Bruising
**Lymphadenopathy  
**[[Lymphadenopathy]]
**Brown recluse spider bites
**[[Brown recluse]] spider bites


==Differential Diagnosis==
==Differential Diagnosis==
===Acquired Hemolytic Anemia===
===Acquired Hemolytic Anemia===
====Microangiopathic Hemolytic Anemia====
====Microangiopathic Hemolytic Anemia====
*HUS
*[[HUS]]
*TTP
*[[TTP]]
*DIC
*[[DIC]]
*Malignant Hypertension
*[[Hypertensive emergency]]
====Autoimmune====
====Autoimmune====
*Warm Antibody Autoimmune Hemolytic Anemia
*[[Autoimmune hemolytic anemia]]
====Infection====
====Infection====
*Malaria
*[[Malaria]]
*Babesia
*[[Babesiosis]]
 
====Other====
====Other====
*Brown recluse spider venom
*[[Brown recluse spider venom]]
===Hereditary/Congenital Hemolytic Anemia===
===Hereditary/Congenital Hemolytic Anemia===
*G6PD – medication induced hemolytic anemia
*[[G6PD]] – medication induced hemolytic anemia
*Sickle Cell Disease
*[[Sickle cell disease]]
*Thalassemia
*[[Thalassemia]]
*Hereditary Spherocytosis
*[[Hereditary spherocytosis]]


==Evaluation==
==Evaluation==
*Labs
===Workup===
** CBC
*CBC
***Look for low h/h
**Low hemaglobin/hematocrit
***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
** 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]] (for hemoglobin, hemosiderin), uHCG
** 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'''
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| align="center" style="background:#f0f0f0;"|'''Thick/Thin Prep'''
| align="center" style="background:#f0f0f0;"|'''Thick/Thin Prep'''
|-
|-
| HUS||low||low||elevated||highly elevated||schistocytes||wnl||elevated||low||wnl||negative||negative
| [[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
| [[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
| [[DIC]]||low||low||elevated||elevated||schistocytes||elevated||elevated||low||low||negative||negative
|-
|-
| Malignant Hypertension||low||low||elevated||variable||schistocytes||||elevated||low||||negative||negative
| Malignant Hypertension||low||low||elevated||variable||schistocytes||||elevated||low||||negative||negative
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| align="center" style="background:#f0f0f0;"|'''Thick/Thin Prep'''
| align="center" style="background:#f0f0f0;"|'''Thick/Thin Prep'''
|-
|-
| Malaria||low||wnl||elevated||||see thick/thin prep||variable||elevated||low||wnl||negative||paracytes
| [[Malaria]]||low||wnl||elevated||||see thick/thin prep||variable||elevated||low||wnl||negative||paracytes
|-
|-
| Babesia||||||||||see thick/thin prep||||||||||negative||paracytes
| Babesia||||||||||see thick/thin prep||||||||||negative||paracytes
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| align="center" style="background:#f0f0f0;"|'''Thick/Thin Prep'''
| align="center" style="background:#f0f0f0;"|'''Thick/Thin Prep'''
|-
|-
| Brown rec||low||wnl||elevated||||||||elevated||||||positive||negative
| [[Brown recluse]]||low||wnl||elevated||||||||elevated||||||positive||negative
|}
|}
==Management ==
 
*ABC’s and Resuscitation if necessary
==Management==
*2 Large bore IVs
*ABC’s and resuscitation if necessary, 2 large bore IVs
*Emergent hematology consultation if patient is very ill appearing
*Emergent hematology consultation if patient is very ill appearing
===Acquired Hemolytic Anemia===
===Acquired Hemolytic Anemia===
====Microangiopathic Hemolytic Anemia====
====Microangiopathic Hemolytic Anemia====
*HUS
*[[HUS]]
**Supportive Care
**Supportive Care
***Hydration
***Hydration
***Pain control
***Pain control
**Hemodialysis if acute renal failure
**Hemodialysis if acute renal failure
**Do NOT give Antibiotics: results in increased expression of Shiga Toxin from E. Coli O157:H7
**Do NOT give antibiotics: results in increased expression of Shiga Toxin from E. Coli O157:H7
 
*[[TTP]]
*TTP
**'''Avoid''' platelet transfusion, except in life-threatening bleeding or intracranial hemorrhage
** Avoid platelet transfusion, except in life-threatening bleeding or intracranial hemorrhage
**[[Plasma exchange]]
**Plasma Exchange
**If Plasma exchange cannot be performed immediately, give FFP and plasmapharese later.
** If Plasma exchange cannot be performed immediately, give FFP and pheresis later.
**Factor VIII concentrate
** Infusion with factor VIII concentrate
*[[DIC]]
 
**Platelet transfusion if count is <50,000 and/or significant bleeding
*DIC
**Platelets if count is <50,000 and/or significant bleeding
**pRBC if active bleeding or hemodynamically unstable
**pRBC if active bleeding or hemodynamically unstable
**FFP if active bleeding
**[[FFP]] if active bleeding
**Cryoprecipitate if fibrinogen <150 and bleeding
**[[Cryoprecipitate]] if fibrinogen <150 and bleeding
**TXA is only indicated for active or massive bleeding
**[[TXA]] is only indicated for active or massive bleeding
 
*[[Malignant hypertension]]
*Malignant hypertension
**Reduce blood pressure as clinically indicated
**Decreased blood pressure as clinically indicated


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


====Infection====
====Infection====
*Malaria
*[[Malaria]]
** Visit CDC website to determine resistance pattern: https://www.cdc.gov/malaria/diagnosis_treatment/treatment.html
**Visit CDC website to determine resistance pattern: https://www.cdc.gov/malaria/diagnosis_treatment/treatment.html
** CDC Malaria Hotline: (770) 488-7788 or (855) 856-4713 toll-free Monday-Friday 9 am to 5 pm EST - (770) 488-7100 after hours, weekends and holidays
**CDC Malaria Hotline: (770) 488-7788 or (855) 856-4713 toll-free Monday-Friday 9 am to 5 pm EST - (770) 488-7100 after hours, weekends and holidays
 
*[[Babesiosis]]
*Babesia
**Mild/moderate Disease
**Mild/moderate Disease
*** Atovaquone 750mg PO q12hr plus Azithromycin 500mg PO on day 1, then 250mg/day PO
***[[Atovaquone]] 750mg PO q12hr '''AND'''
***[[Azithromycin]] 500mg PO on day 1, then 250mg/day PO
**Severe Disease
**Severe Disease
*** Adult: Clindamycin 300-600mg IV qid or 600mg PO TID Plus Quinine 650mg PO TID x 7-10 days
***[[Clindamycin]] 300-600mg IV QID '''OR''' 600mg PO TID '''AND'''
***[[Quinine]] 650mg PO TID x7-10 days
***Consider exchange transfusion if parasitemia >10%
***Consider exchange transfusion if parasitemia >10%
**Supportive treatment
**Supportive treatment


====Other====
====Other====
*Brown recluse spider venom
*[[Brown recluse spider bite]]
**Supportive care
**Supportive care
***Hemodynamic support with fluids and pressers if necessary
***Hemodynamic support with fluids and pressers if necessary
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===Hereditary/Congenital Hemolytic Anemia===
===Hereditary/Congenital Hemolytic Anemia===
*G6PD
*[[G6PD]]
**Stop new medications
**Stop new medications
**Treat any infections aggressively
**Treat any infections aggressively
**Ovoid oxidant drugs
**Ovoid oxidant drugs
**Blood transfusion if severe illness
**Blood transfusion if severe illness
 
*[[Sickle Cell Disease]], [[Thalassemia]], [[Hereditary spherocytosis]]
*Sickle Cell Disease
**Hemolytic anemia in the above diseases typically chronic, usually does not require treatment in ED
**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==
==Disposition==
Admit
*Depends on severity, complications, etc.


==Also See==
==See Also==
*[[Anemia]]


==External Links==
==External Links==


==References==
==References==
*Dhaliwal G, Cornett P, Tierney LM Jr. Hemolytic Anemia. Am Fam Physician. 2004 Jun 1;69(11):2599-606.
<references/>
* 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.
[[Category:Heme/Onc]]
*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

Latest revision as of 03:09, 14 December 2020

Background

  • Wide variety of clinical presentation given the large differential diagnosis
  • Clinical presentation and lab findings differ depending on intravascular vs. extravascular hemolysis, acute vs. chronic
  • Common findings
    • low hemoglobin and hematocrit
    • reticulocytosis
    • elevated indirect bilirubin
  • Most important lab to elucidate diagnosis is blood smear
  • Most common emergent presentations are due to acute intravascular hemolytic anemias

Etiologies

Divided by etiology: acquired vs. hereditary

Clinical Features

History

PMH

Physical Exam

Differential Diagnosis

Acquired Hemolytic Anemia

Microangiopathic Hemolytic Anemia

Autoimmune

Infection

Other

Hereditary/Congenital Hemolytic Anemia

Evaluation

Workup

  • CBC
    • Low hemaglobin/hematocrit
    • Low platelet count → microangiopathic hemolytic anemia
  • Blood smear
  • Reticulocyte count
  • CMP
    • Most important: indirect bilirubin and creatinine
  • UA (for hemoglobin, hemosiderin), uHCG
  • 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 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 recluse 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 plasmapharese later.
    • Factor VIII concentrate
  • DIC
    • Platelet transfusion 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
    • Reduce 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

Other

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

Hereditary/Congenital Hemolytic Anemia

Disposition

  • Depends on severity, complications, etc.

See Also

External Links

References

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