Dive into a gripping pediatric ICU case featuring a seven-year-old girl with hemoglobin SC disease. The hosts break down the vital anatomy of the spleen and its critical role in immunity, especially in sickle cell patients. Discover the challenges posed by acute splenic sequestration, from symptoms to diagnostics. Learn about effective management strategies, the importance of blood transfusions, and preventive care measures. This discussion is packed with essential clinical insights for supporting young patients through complex health crises!
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insights INSIGHT
Spleen Is A High‑Throughput Blood Filter
The spleen filters blood and acts as a reservoir for RBCs and platelets while defending against blood-borne pathogens.
Red pulp clears abnormal RBCs via slow transit and macrophage-mediated erythrophagocytosis at narrow sinus slits.
insights INSIGHT
White Pulp Drives Anti‑Encapsulated Bacterial Immunity
White pulp organizes B and T cells to initiate immune responses to blood-borne antigens, especially encapsulated bacteria.
Follicular B cells mediate T-dependent responses while mantle zone memory B cells trigger T-independent responses.
insights INSIGHT
Perifollicular Zone Matures After Infancy
The perifollicular zone links red and white pulp and its macrophages develop around age two.
Immature perifollicular zones explain early childhood susceptibility to pneumococcal infections.
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Ready for a deep dive into a real-life pediatric ICU situation? Today, Dr. Pradip Kamat, Dr. Monica Gray, and Dr. Rahul Damania will walk you through the case of a seven-year-old girl with Hemoglobin SC (HbSC) disease, who presents with abdominal swelling, pneumonia, low oxygen, and pain.
In this episode, our team unpacks the spleen’s anatomy and its crucial role in immunity, then zooms in on how sickle cell disease can throw a wrench in splenic function. You’ll hear how they approach the diagnosis and management of acute splenic sequestration crisis, sharing clinical pearls along the way. Plus, they’ll break down why quick recognition is so important and discuss strategies for both immediate and long-term care in pediatric sickle cell patients. Don’t miss these practical insights from the frontlines of pediatric critical care!
Show Highlights:
Case study of a seven-year-old girl with hemoglobin SC disease
Presentation of symptoms: abdominal distension, pneumonia, hypoxia, and body pain
Discussion of acute splenic sequestration crisis as a complication of sickle cell disease
Anatomy and physiology of the spleen
The role of the spleen in sickle cell disease and how sickled cells affect splenic function
Acute splenic sequestration crisis, including clinical features and laboratory evaluations
Management strategies for acute splenic sequestration crisis in the ICU
Importance of blood transfusions and supportive care in treatment
Prophylactic measures to prevent recurrence of splenic sequestration
Educational emphasis on recognizing clinical signs and the need for timely intervention
References:
Fuhrman & Zimmerman - Textbook of Pediatric Critical Care Chapter 88. Hemoglobinopathies. Baender, MA, Marsh Anne. Pages: 1457-1470
Rogers' textbook of pediatric intensive care: Hematologic Emergencies. McCory MC, Bhar S, and Blaine E. Pages 2003-2005
Brousse V, Buffet P, Rees D. The spleen and sickle cell disease: the sick(led) spleen. Br J Haematol. 2014 Jul;166(2):165-76. doi: 10.1111/bjh 12950. Epub 2014 May 26. PMID: 24862308.
Waleed S, Aldabsa M, Gouher S. Splenic Sequestration Induced by Parvovirus B19: A Case Report. Cureus. 2024 May 23;16(5):e60937. doi: 10.7759/cureus. 60937. PMID: 38915956; PMCID: PMC11195323.
Solanki DL, Kletter GG, Castro O. Acute splenic sequestration crises in adults with sickle cell disease. Am J Med. 1986 May;80(5):985-90. doi: 10.1016/0002-9343(86)90649-2. PMID: 3706382.
Karna B, Jha SK, Al Zaabi E. Hemoglobin C Disease. [Updated 2023 May 29]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK559043/
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