Age: 5.0
Sex: M
Vaccine: PPV
Symptoms: Lethargy, Oxygen saturation normal, Pain in extremity, Pallor, Platelet count normal
Clinical narrative: Information has been received from the author of a published article. It was reported that, "a 5-year-old black male with sickle B - thalassemia born to a mother with sickle cell trait and a father with B-thalassemia trait was admitted to the hospital for fever and pain of the left arm and back. He had a history of 2 prior admissions: one for moderately severe vaso-occlusive crisis of the legs and another for fever of unknown origin. He received Pneumovax 23 at the age of 2 years and was on folic acid oral supplementation. There was no history of acute chest syndrome or splenic sequestration and no history of blood transfusions. Review of his medical records showed a baseline hemoglobin level of 7.5 (+/- 0.6) g/dL and a pretransfusion hemoglobin electrophoresis showed hemoglobin S >82 percent; F 12 percent to 16 percent; A2 3.9 percent to 7,8 percent; and no detectable A1. At presentation, the child was febrile to 39 degrees C with an oxygen saturation of 98 percent on room air by pulse oximetry. The physical examination showed erythema of the oropharynx, and notably no hepatomegaly was appreciable by either the admitting or the inpatient physician. Pain was assessed as 6 on a scale of 1 to 10, thus morphine sulfate was given intravenously and acetaminophen orally. Hemoglobin level was 7.1 g/dL, hematocrit was 21.6 percent, total white blood cell count was 16,700/ uL, and platelet count was 115,000/uL. A chest x-ray was normal. Ceftriaxone 50 mg/kg was given intravenously after blood cultures were collected. Four hours after admission, he complained of a severe headache, was found to be more lethargic, toxic looking, and had a persistent tachycardia and tachypnea. His lips were pale. The abdomen was slightly distended in comparison with his initial assessment but soft and firm liver edge was palpable 4 cm below the costal margin in the mid-clavicular line. A blood sample showed profound anemia (hemoglobin 3.2 g/dl, repeat in 30 min was 2.1 g/dL) and thrombocytopenia (17,000/uL) with a drop in the initial white count to 4600/uL. Ceftriaxone-induced acute hemolysis was considered and ruled out with the appropriate testing. Packed red blood cell (PRBC) transfusion was started promptly. The hemoglobin levels dropped rapidly after each transfusion. The liver enlarged progressively, and within 2 hours is spanned 8 cm in the right mid-clavicular line. Liver enzymes were 12-fold increased whereas bilirubin measured 4 mg/dL (mostly conjugated). An abdominal ultrasound showed massive hepatomegaly (14 cm vertical span), thickened gallbladder wall, and edema of the bowel loops. Doppler study of the liver showed no detectable hepatic vein, portal vein, or hepatic arterial blood flows. The inferior vena cava maintained a good flow. The patient subsequently became hemodynamically unstable and needed cardiorespiratory support in addition to repeated transfusion of PRBC, platelets, and fresh frozen plasma. This picture was consistent with sepsis. After 14 hours of incubation, the blood culture grew gram-positive diplococci, later identified as S. pneumoniae serotype 13. Although the hemoglobin level seem to plateau above 12 g/dL, the patient's condition worsened progressively with maximal cardiorespiratory support, massive hepatomegaly (12 cm below the costal margin in the mid-clavicular line), and a very tense abdominal wall. He died 18 hours after admission. Postmortem examination was performed. The liver was significantly enlarged weighing 1089 g. almost twice the normal weight for age (normal weight for age = 596 g), with a red smooth homogenous surface and focal areas of necrosis. Microscopic examination showed markedly dilated sinusoids packed with red blood cells the majority of which were sickle-shaped. However, a fair number of normal erythrocytes were also present, probably donor RBCs form blood transfusions. The spleen weighted only 20.5 g (normal weight for age = 47 g). All other organs examined were congested and hemorrhagic, consistent with terminal disseminated-intravascular-thrombosis. The authors reported that, "hepatic sequestration was preceded by pneumococcal sepsis. The role of the pneumococcal bacteremia as a possible triggering factor for the sequestration process and as a major contributor to the irreversible hemodynamic decompensation should be taken into consideration." No further information is available. A copy of the published article is attached as further documentation of the patient's experience.