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    Subjects/Pathology/Sickle Cell Disease
    Sickle Cell Disease
    medium
    microscope Pathology

    A 7-year-old boy with sickle cell disease presents with acute chest pain, fever, and cough. Chest X-ray shows a new infiltrate. All of the following are recognized complications of sickle cell disease EXCEPT:

    A. Proliferative diabetic retinopathy as the most common ocular complication
    B. Acute chest syndrome with pulmonary infarction and/or infection
    C. Osteomyelitis predominantly caused by Salmonella species
    D. Splenic sequestration crisis with rapid splenomegaly and hypovolemic shock

    Explanation

    Complications of Sickle Cell Disease

    Acute Chest Syndrome (ACS)
    Key Point
    ACS is a life-threatening complication characterized by chest pain, fever, cough, and new pulmonary infiltrates. It results from pulmonary infarction (due to vaso-occlusion), infection (pneumonia), or fat embolism from bone marrow infarction. It is a leading cause of death in sickle cell disease.
    Splenic Sequestration Crisis
    High-YieldNEET PG
    Splenic sequestration occurs when sickled RBCs are trapped in the spleen, causing rapid splenomegaly, acute anemia, thrombocytopenia, and hypovolemic shock. This is a medical emergency requiring urgent transfusion. It typically occurs in children under 5 years and is rare in adults (due to autoinfarction of the spleen).
    Osteomyelitis in Sickle Cell Disease
    Clinical Pearl
    Patients with sickle cell disease have a 50-fold increased risk of osteomyelitis. Salmonella species are the most common causative organisms (unlike the general population where Staphylococcus aureus predominates). This is a classic association tested in NEET PG.
    Mnemonic
    SALMONELLA in SICKLE CELL — both start with 'S'.
    Ocular Complications
    Warning
    The most common ocular complication of sickle cell disease is proliferative sickle retinopathy (not diabetic retinopathy). Proliferative sickle retinopathy results from retinal vaso-occlusion and neovascularization, leading to "sea fan" neovascularization and potential retinal detachment. Diabetic retinopathy is a complication of diabetes mellitus, not sickle cell disease.
    Table
    ComplicationMechanismClinical Feature
    Acute chest syndromePulmonary infarction ± infectionChest pain, fever, infiltrate
    Splenic sequestrationRBC trapping in spleenAcute anemia, splenomegaly, shock
    OsteomyelitisSalmonella (classically)Bone pain, fever
    Proliferative retinopathyRetinal vaso-occlusionSea fan neovascularization
    Diabetic retinopathyHyperglycemia (diabetes)NOT a sickle cell complication
    High-YieldNEET PG
    Proliferative sickle retinopathy, not diabetic retinopathy, is the characteristic ocular finding in sickle cell disease.

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