Case history
Case history #1
A 23-year-old man of Iranian origin consults his primary care provider about a skin rash. He has an intensely pruritic rash over his buttocks and back that has appeared spontaneously. He is referred to a dermatologist, who diagnoses dermatitis herpetiformis and prescribes dapsone. Two days later he develops severe nausea and exhaustion, and complains of passing dark urine. His wife notices he has become jaundiced. He attends the ER, where investigations reveal anemia (Hb 7.5 g/dL) and abnormal biochemistry (elevated BUN, deranged liver function, and unconjugated hyperbilirubinemia). An urgent hematology consult is obtained and a diagnosis of drug-induced hemolytic anemia made.
Case history #2
An 18-year-old Greek man presents to the ER with severe nausea, vomiting, and diarrhea. His mother explains that he had been at a lunch party with friends and none of the other guests were ill. The patient had ingested a meal of rice, meat, and freshly cooked beans. He has not had any significant illnesses in the past. Examination reveals a markedly dehydrated young man who is clinically anemic and jaundiced. Investigations show a hemoglobin of 5.1 g/dL, elevated WBC count with a predominant neutrophilia, elevated BUN and creatinine, and deranged liver function. No urine can be obtained. Intravenous fluids are commenced, followed by a transfusion of packed red cells; the patient becomes acutely dyspneic, however, and chest x-ray shows features of pulmonary edema. A nephrologist is consulted. Intravenous diuretic therapy is prescribed, a urinary catheter inserted, and 30 mL of urine obtained that, on testing, shows a high urobilinogen and protein content.
Other presentations
Patients (usually boys) are at an increased risk of developing neonatal jaundice. This usually presents 2 to 3 days after birth as exaggerated physiologic jaundice with minimal anemia. However, in some cases the jaundice and anemia are severe, and treatment with exchange blood transfusion and phototherapy may be required. Hemolytic anemia can also occur (also typically in males) in the setting of the recovery phase of an acute infection (bacterial or viral; typically pneumonia or typhoid fever). A few patients (exclusively males) with severe deficiency enzyme variants have severe neonatal jaundice and chronic hemolytic anemia with splenomegaly. Females are usually asymptomatic but may develop mild drug-induced jaundice.
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