Skip to content
  • About
  • Contact
  • Contribute
  • Book
  • Careers
  • Podcast
  • Recommended
  • Speaking
  • All
  • Physician
  • Practice
  • Policy
  • Finance
  • Conditions
  • .edu
  • Patient
  • Meds
  • Tech
  • Social
  • Video
    • All
    • Physician
    • Practice
    • Policy
    • Finance
    • Conditions
    • .edu
    • Patient
    • Meds
    • Tech
    • Social
    • Video
    • About
    • Contact
    • Contribute
    • Book
    • Careers
    • Podcast
    • Recommended
    • Speaking

MKSAP: 24-year-old woman with sickle cell anemia

mksap
Conditions
January 21, 2017
247 Shares
Share
Tweet
Share

Test your medicine knowledge with the MKSAP challenge, in partnership with the American College of Physicians.

A 24-year-old woman undergoes routine evaluation. She is pregnant at 12 weeks’ gestation. Medical history is notable for homozygous sickle cell anemia (Hb SS). She has had multiple uncomplicated painful crises treated at home with hydration, nonopioid analgesia, and incentive spirometry. She requires hospital management for these episodes approximately twice per year. She has declined the use of hydroxyurea. Her only other medication is folic acid.

On physical examination, vital signs are normal. Mild scleral icterus is noted. A grade 2/6 early systolic flow murmur is heard at the cardiac base. The examination is otherwise normal. Laboratory results show a hemoglobin level of 7.5 g/dL (75 g/L).

Which of the following is the most appropriate management?

A: Erythrocyte transfusion to maintain hemoglobin level at 10 g/dL (100 g/L)
B: Erythropoiesis-stimulating agent
C: Exchange transfusion
D: No transfusion at this time

MKSAP Answer and Critique

The correct answer is D: No transfusion at this time.

The patient should not receive an erythrocyte transfusion at this time. Erythrocyte transfusion in sickle cell disease (SCD) is appropriate only for specific indications, including stroke, symptomatic anemia, acute chest syndrome (ACS), surgical interventions, secondary prevention of stroke or ACS, and, possibly, prevention of priapism, pulmonary hypertension, and nonhealing ulcers. Transfusion is not indicated for uncomplicated pregnancy, routine painful episodes, minor surgery not requiring anesthesia, or asymptomatic anemia. Erythrocyte exchange transfusion is indicated for acute ischemic stroke, ACS with significant hypoxia, and multiorgan failure/hepatopathy as well as in persons in whom simple transfusion would increase the hemoglobin level to greater than 10 g/dL (100 g/L). Chronic transfusion can lead to iron overload, alloimmunization, and an increased risk for a delayed hemolytic transfusion reaction. Erythrocytes used in transfusion should be leukoreduced, hemoglobin S negative, and phenotypically matched for the E, C, and K antigens as well as for any known alloantibodies. Hemoglobin targets should remain less than 10 g/dL (100 g/L) to avoid hyperviscosity.

In a randomized trial, transfusion reduced the risk of pain crisis in pregnant women with SCD but showed no clear improvement in maternal mortality, perinatal mortality, or severe maternal morbidity (pulmonary embolism, chronic heart failure, ACS). Transfusion should be provided based on symptoms of anemia and not hemoglobin levels.

Erythropoiesis-stimulating agents (ESAs) are used to treat anemia in conditions in which bone marrow stimulation of erythrocyte production is inadequate, such as chronic kidney disease. However, in SCD, erythropoietin levels are typically high to augment bone marrow erythrocyte production in response to chronic hemolysis. Therefore, ESAs are not indicated for treatment of the anemia associated with SCD.

Key Point

  • In patients with sickle cell disease, including pregnant patients, transfusion is not indicated for uncomplicated pregnancy, routine painful episodes, minor surgery not requiring anesthesia, or asymptomatic anemia.

This content is excerpted from MKSAP 17 with permission from the American College of Physicians (ACP). Use is restricted in the same manner as that defined in the MKSAP 16 Digital license agreement. This material should never be used as a substitute for clinical judgment and does not represent an official position of ACP. All content is licensed to KevinMD.com on an “AS IS” basis without any warranty of any nature. The publisher, ACP, shall not be liable for any damage or loss of any kind arising out of or resulting from use of content, regardless of whether such liability is based in tort, contract or otherwise.

Prev

I demand you consider us humans: A medical student poem

January 20, 2017 Kevin 7
…
Next

Confessions of a former TV doctor

January 21, 2017 Kevin 0
…

Tagged as: Hematology

Post navigation

< Previous Post
I demand you consider us humans: A medical student poem
Next Post >
Confessions of a former TV doctor

More by mksap

  • a desk with keyboard and ipad with the kevinmd logo

    MKSAP: 26-year-old man with back pain

    mksap
  • a desk with keyboard and ipad with the kevinmd logo

    MKSAP: 36-year-old man with abdominal cramping, diarrhea, malaise, and nausea

    mksap
  • a desk with keyboard and ipad with the kevinmd logo

    MKSAP: 52-year-old woman with osteoarthritis of the right hip

    mksap

Related Posts

  • How sickle cell anemia influences a medical school journey

    Bianca Bowden
  • a desk with keyboard and ipad with the kevinmd logo

    MKSAP: 35-year-old woman with constipation

    mksap
  • a desk with keyboard and ipad with the kevinmd logo

    MKSAP: 60-year-old woman with persistent constipation

    mksap
  • a desk with keyboard and ipad with the kevinmd logo

    MKSAP: 45-year-old woman with type 2 diabetes mellitus

    mksap
  • 3 ways we’ve failed woman who breastfeed

    Joanna Buscemi, PhD
  • How one woman prevented a pharmaceutical disaster

    James Essinger and Sandra Koutzenko

More in Conditions

  • A teenager’s perspective: the pressing need for mental health days in schools

    Ruhi Saldanha
  • The art of pediatrics: Connecting through observation

    Alexander Rakowsky, MD
  • Epigenetics and our inheritance to future generations

    Vishruth Nagam
  • COVID-19 unleashed an ongoing crisis of delirium in hospitals

    Christina Reppas-Rindlisbacher, MD, Nathan Stall, MD, and Paula Rochon, MD
  • Emergency care nightmare: the urgent need for experienced nurses

    Rachel Basham, RN, CCRN
  • Debating the role of psychiatric assessments in medical decisions

    Christian Youssef & Francisco M. Torres, MD
  • Most Popular

  • Past Week

    • The erosion of patient care

      Laura de la Torre, MD | Physician
    • Reigniting after burnout: 3 physician stories

      Kim Downey, PT | Physician
    • Inside the grueling life of a surgery intern

      Randall S. Fong, MD | Physician
    • A teenager’s perspective: the pressing need for mental health days in schools

      Ruhi Saldanha | Conditions
    • Challenging the diagnosis: dehydration or bias?

      Sydney Lou Bonnick, MD | Physician
    • Exploring HIV care and advocacy [PODCAST]

      The Podcast by KevinMD | Podcast
  • Past 6 Months

    • Medical gaslighting: a growing challenge in today’s medical landscape

      Tami Burdick | Conditions
    • I want to be a doctor who can provide care for women: What states must I rule out for my medical education?

      Nandini Erodula | Education
    • Balancing opioid medication in chronic pain

      L. Joseph Parker, MD | Conditions
    • Mourning the silent epidemic: the physician suicide crisis and suggestions for change

      Amna Shabbir, MD | Physician
    • Reigniting after burnout: 3 physician stories

      Kim Downey, PT | Physician
    • I’m a doctor, and I almost died during childbirth

      Bayo Curry-Winchell, MD | Physician
  • Recent Posts

    • Exploring HIV care and advocacy [PODCAST]

      The Podcast by KevinMD | Podcast
    • A teenager’s perspective: the pressing need for mental health days in schools

      Ruhi Saldanha | Conditions
    • Challenging the diagnosis: dehydration or bias?

      Sydney Lou Bonnick, MD | Physician
    • The art of pediatrics: Connecting through observation

      Alexander Rakowsky, MD | Conditions
    • Assertiveness in health care [PODCAST]

      The Podcast by KevinMD | Podcast
    • Epigenetics and our inheritance to future generations

      Vishruth Nagam | Conditions

Subscribe to KevinMD and never miss a story!

Get free updates delivered free to your inbox.


Find jobs at
Careers by KevinMD.com

Search thousands of physician, PA, NP, and CRNA jobs now.

Learn more

View 2 Comments >

Founded in 2004 by Kevin Pho, MD, KevinMD.com is the web’s leading platform where physicians, advanced practitioners, nurses, medical students, and patients share their insight and tell their stories.

Social

  • Like on Facebook
  • Follow on Twitter
  • Connect on Linkedin
  • Subscribe on Youtube
  • Instagram

CME Spotlights

From MedPage Today

Latest News

  • Lab Tests That Escape FDA Oversight May Come Under Agency Review
  • Fezolinetant Benefits Women Not Suited for Hormone Therapy
  • Low Tidal Volume Compliance Still Lacking in Mechanical Ventilation
  • IV Immunoglobulin May Cut Infection Risk of Anti-BCMA Agents for Myeloma
  • When's the Best Time to Get the Updated COVID Shot?

Meeting Coverage

  • Fezolinetant Benefits Women Not Suited for Hormone Therapy
  • Plant-Based Estrogen Improves Lipids in Postmenopausal Women
  • New Schizophrenia Treatments Are Coming: Don't Panic
  • Loneliness Needs to Be Treated Like Any Other Health Condition, Researcher Suggests
  • Stopping Medical Misinformation Requires Early Detection
  • Most Popular

  • Past Week

    • The erosion of patient care

      Laura de la Torre, MD | Physician
    • Reigniting after burnout: 3 physician stories

      Kim Downey, PT | Physician
    • Inside the grueling life of a surgery intern

      Randall S. Fong, MD | Physician
    • A teenager’s perspective: the pressing need for mental health days in schools

      Ruhi Saldanha | Conditions
    • Challenging the diagnosis: dehydration or bias?

      Sydney Lou Bonnick, MD | Physician
    • Exploring HIV care and advocacy [PODCAST]

      The Podcast by KevinMD | Podcast
  • Past 6 Months

    • Medical gaslighting: a growing challenge in today’s medical landscape

      Tami Burdick | Conditions
    • I want to be a doctor who can provide care for women: What states must I rule out for my medical education?

      Nandini Erodula | Education
    • Balancing opioid medication in chronic pain

      L. Joseph Parker, MD | Conditions
    • Mourning the silent epidemic: the physician suicide crisis and suggestions for change

      Amna Shabbir, MD | Physician
    • Reigniting after burnout: 3 physician stories

      Kim Downey, PT | Physician
    • I’m a doctor, and I almost died during childbirth

      Bayo Curry-Winchell, MD | Physician
  • Recent Posts

    • Exploring HIV care and advocacy [PODCAST]

      The Podcast by KevinMD | Podcast
    • A teenager’s perspective: the pressing need for mental health days in schools

      Ruhi Saldanha | Conditions
    • Challenging the diagnosis: dehydration or bias?

      Sydney Lou Bonnick, MD | Physician
    • The art of pediatrics: Connecting through observation

      Alexander Rakowsky, MD | Conditions
    • Assertiveness in health care [PODCAST]

      The Podcast by KevinMD | Podcast
    • Epigenetics and our inheritance to future generations

      Vishruth Nagam | Conditions

MedPage Today Professional

An Everyday Health Property Medpage Today
  • Terms of Use | Disclaimer
  • Privacy Policy
  • DMCA Policy
All Content © KevinMD, LLC
Site by Outthink Group

MKSAP: 24-year-old woman with sickle cell anemia
2 comments

Comments are moderated before they are published. Please read the comment policy.

Loading Comments...