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Anemia, Sideroblastic

Abdulaziz Almedimigh, MBBS, Anna L. Silverman, MD and Marie L. Borum, MD, EdD, MPH Reviewed 06/2018
 


BASICS

DESCRIPTION

  • Sideroblastic anemia (SA) is a disorder of iron utilization in the erythroblast resulting in ineffective erythropoiesis and variable systemic iron overload leading to anemia. It is c...

DIAGNOSIS

Moderate to severe anemia with fatigue, dizziness, and dyspnea should prompt an evaluation for SA. 

HISTORY

  • Symptoms of anemia, including fatigue, dizziness, and dyspnea (3)[A]

  • Alcohol or drug ...

TREATMENT

MEDICATION

First Line

  • Treatment is largely supportive (2)[A].

  • Pyridoxine

    • Will improve anemia only in X-linked SA and alcohol or isoniazid-related SA (2)[A]

    • Initial dose should be 50 to 100 mg PO ...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Annual ferritin and transferrin saturation to monitor for iron overload (3)[A]

  • Patients given pyridoxine should be followed for response to treat...

REFERENCES

1
Bottomley SS, Fleming MD. Sideroblastic anemia: diagnosis and management. Hematol Oncol Clin North Am.  2014;28(4):653–670. {L-End} [View Abstract on OvidMedline]
2
Camaschella C. Here...

ADDITIONAL READING

  • Cuijpers ML, van Spronsen DJ, Muus P, et al. Need for early recognition and therapeutic guidelines of congenital sideroblastic anaemia. Int J Hematol.  2011;94(1):97–100.

  • Müller...

SEE ALSO

Algorithm: Anemia 

CODES

ICD10

  • D64.3 Other sideroblastic anemias

  • D64.0 Hereditary sideroblastic anemia

  • D64.1 Secondary sideroblastic anemia due to disease

  • D64.2 Secondary sideroblastic anemia due to drugs and toxins

ICD9

285....

CLINICAL PEARLS

  • SA results from inability to incorporate iron during heme synthesis in the mitochondria, leading to microcytic anemia and sideroblasts in the bone marrow.

  • Multiple inherited forms are l...

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