Pathology · Anemias (Hemolytic, Microcytic, Macrocytic, Hemoglobinopathies)

A 5-year-old boy develops dark red urine two days after a febrile upper respiratory infection. Hemoglobin is 7 g/dL with a reticulocyte count of 0.5 percent. Direct antiglobulin test is positive for complement only. Incubation of his serum with normal RBCs causes lysis at 4 degrees Celsius and again on rewarming to 37 degrees Celsius. The responsible antibody has specificity against which antigen?

  • A I antigen
  • B Kell antigen
  • C Rh D antigen
  • D P antigen
Correct answer: D. P antigen

Explanation

This is paroxysmal cold hemoglobinuria. The Donath-Landsteiner antibody is an IgG biphasic hemolysin that binds the P antigen on red cells in the cold peripheral circulation, fixes complement, and then lyses the cells when they rewarm centrally. Cold agglutinin disease antibodies are IgM directed against the I or i antigen and cause chronic extravascular hemolysis rather than episodic hemoglobinuria. PCH typically follows viral infections in children and usually resolves spontaneously within weeks.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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