'Sequestration crisis' is related to:

AIIMS Raipur NO - 2019 (Shift-1)

Medical & Surgical Nursing

Easy

Appeared in: AIIMS Raipur NO - 2019 (Shift-1)

Explanation

A sequestration crisis is a hallmark complication of sickle cell disease.
It occurs when sickled red blood cells obstruct blood flow out of the spleen, causing a large volume of blood to be trapped (sequestered) there.
This leads to a rapid, severe drop in hemoglobin and circulating blood volume, presenting as acute anemia and hypovolemic shock.
This phenomenon is unique to conditions with abnormally shaped or rigid red cells, like sickle cell anemia, and is not a feature of anemias caused by nutritional deficiencies or bone marrow failure.

Option A: Pernicious anemia is caused by a deficiency in vitamin B12 due to a lack of intrinsic factor. It affects the maturation of red blood cells in the bone marrow but does not cause them to sickle or become trapped in the spleen.
Option C: Aplastic anemia is a rare condition where the bone marrow fails to produce sufficient numbers of all blood cell types (pancytopenia). The problem is one of production, not the shape or function of the cells that are produced.
Option D: Iron deficiency anemia is the most common type of anemia, caused by a lack of iron. This leads to the production of small (microcytic) and pale (hypochromic) red blood cells, but it does not cause the sickling shape that leads to sequestration.

Clinical Relevance

Nursing practice connection: This is primarily an exam-oriented knowledge point with limited direct bedside application, so retain Complications of Sickle Cell Anemia as background academic context rather than a clinical decision trigger.
Splenic sequestration is a life-threatening pediatric emergency. Nurses must be able to rapidly assess for signs like sudden pallor, tachycardia, and abdominal distension (enlarged spleen) in children with sickle cell disease.
Patient and family education is a key nursing role. Teaching parents how to palpate for the spleen and when to seek immediate medical attention can be life-saving.
What if? If the patient was an adult with sickle cell disease, a sequestration crisis would be much less likely. Due to repeated sickling and infarction, most adults experience 'autosplenectomy' (a shrunken, non-functional spleen) by adulthood, making massive sequestration impossible. In adults, the liver can sometimes be a site of sequestration.

How to Approach the Question

First, identify the key term in the question: 'Sequestration crisis'.
Recall or deduce the meaning of 'sequestration' - which means to trap or set apart.
Consider the pathophysiology of each anemia listed in the options.
Evaluate which condition involves red blood cells that are prone to being trapped. Sickle cell anemia is characterized by abnormally shaped, rigid cells that can easily block blood vessels.
Connect the concept of cell trapping (sequestration) with the pathophysiology of sickled cells getting stuck in the spleen's microcirculation.
Rule out the other options as their mechanisms (nutritional deficiency, bone marrow failure) do not involve cell trapping.

Concept Tested & Keywords

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E6 Ghai Essential Pediatrics(pp 26-904 of 913) p. 364-366

E6 Pathology- ROBBINS & COTRAN PATHOLOGIC BASIS OF DISEASE 10TH Ed p. 645-647