A ten-year-old boy was brought to the OPD with fever and swelling of hands after playing football. He gives a history of previous episodes of swelling of hands. On imaging, his spleen was found to be shrunken. Which of the following is a likely diagnosis?

Correct Answer: B. Sickle cell anaemia

The clinical presentation of a 10-year-old boy with fever, swelling of hands (dactylitis), recurrent episodes of swelling, and a shrunken spleen is pathognomonic for sickle cell anaemia. Dactylitis (hand-foot syndrome) is a hallmark acute vaso-occlusive crisis in children with sickle cell disease, typically occurring between ages 6 months and 4 years but can persist into later childhood. The recurrent nature of swelling episodes indicates chronic vaso-occlusive disease. The shrunken (autoinfarction) spleen is a critical discriminator—repeated splenic infarctions from sickling lead to functional asplenia and eventual splenic atrophy, a pathognomonic finding in sickle cell anaemia. This is particularly relevant in the Indian context where sickle cell disease is endemic in tribal populations of central and eastern India (Chhattisgarh, Jharkhand, Odisha). The fever accompanying the swelling suggests an acute crisis, possibly triggered by infection or dehydration. The combination of dactylitis + splenic atrophy + recurrent episodes makes sickle cell anaemia the only diagnosis that fits all clinical features.

Why the other options are wrong

A. Measles — Measles presents with fever, rash (maculopapular), cough, and coryza (3 Cs), not hand-foot swelling or dactylitis. Measles does not cause splenic atrophy; it may cause hepatosplenomegaly acutely. The recurrent swelling episodes and shrunken spleen are incompatible with measles, which is an acute self-limited viral illness. C. Pancreatitis — Pancreatitis presents with epigastric pain, elevated amylase/lipase, and abdominal tenderness—not hand-foot swelling or dactylitis. Pancreatitis does not cause splenic atrophy. While acute pancreatitis can occur in sickle cell disease as a complication, it is not the primary diagnosis here; the clinical picture points to vaso-occlusive crisis with dactylitis. D. Malaria — Malaria presents with fever, chills, and hepatosplenomegaly (enlarged spleen), not splenic atrophy. Malaria does not cause dactylitis or recurrent hand-foot swelling. While malaria is endemic in India and fever is a common presentation, the shrunken spleen and specific pattern of hand swelling exclude malaria as the diagnosis.

High-Yield Facts

Mnemonics

SICKLE CELL CRISIS TRIGGERS CHIS: Cold, Hypoxia, Infection, Stress (dehydration). Any of these can precipitate acute vaso-occlusive crisis in sickle cell patients. SPLENIC COMPLICATIONS IN SICKLE CELL ASHEN SPLEEN: Autoinfarction → Splenic atrophy → Hyposplenism → Encapsulated organism infections → Necrosis (functional asplenia). Remember: shrunken spleen = sickle cell.

NBE Trap

NBE may pair fever with malaria or measles to distract from the specific finding of splenic atrophy, which is pathognomonic for sickle cell disease. The recurrent hand swelling (dactylitis) is the key discriminator that rules out acute infections.

Clinical Pearl

In Indian tribal populations, sickle cell disease screening is recommended in endemic areas. A child presenting with recurrent hand-foot swelling and fever should prompt immediate hemoglobin electrophoresis and peripheral smear examination for sickle cells. Early diagnosis and penicillin prophylaxis (due to asplenia) can prevent life-threatening infections and reduce morbidity.

_Reference: Robbins & Cotran Pathologic Basis of Disease, Ch. 14 (Red Blood Cell Disorders); Harrison's Principles of Internal Medicine, Ch. 104 (Hemoglobinopathies); OP Ghai Textbook of Pediatrics, Ch. on Hemolytic Anaemias_