Case Presentation: A 34 year-old female with a history of anxiety and depression initially presented to an outside hospital and then was transferred to our facility for jaundice. She had given birth 2 months prior to presentation to a healthy child; however, the pregnancy was complicated by severe preeclampsia. On exam, she had a high-normal temperate of 37.9C, heart rate in the 120s, and normal blood pressure. She had scleral icterus, but no abdominal tenderness or distention. There was no exam evidence of hepatomegaly or splenomegaly.Her hemoglobin was initially 8.3 and downtrended to a nadir of 2.7; platelets were 147 with a nadir of 7. Total bilirubin peaked at 35 and direct bilirubin peaked at 21; AST and ALT were initially normal but had a delayed peak of 963 and 411 respectively. Ferritin was 16,500. EBV and respiratory viral panel PCR were negative. DAT was positive. Peripheral smear showed evidence of extravascular hemolysis and atypical lymphocytosis without schistocytes. CT angiography chest/abdomen/pelvis revealed hepatosplenomegaly but was otherwise unrevealing. Liver ultrasound did not show cholecystitis or biliary ductal dilation. Patient suffered from severe anemia refractory to multiple transfusions along with AMS and lactic acidosis. She received Berinert (C1 esterase Inhibitor) for cold agglutinin anemia given her positive DAT. LDH and haptoglobin downtrended, however she had persistent cytopenias, fevers, hepatosplenomegaly, and elevated ferritin. She was empirically treated for hemophagocytic lymphocytic histiocytosis (HLH) with dexamethasone and etopside. Her symptoms, hemodynamic parameters and laboratory markers all subsequently improved.
Discussion: HLH is a condition involving a hyperinflammatory state with dysregulation of macrophages, NK cells, and T-cells. It is associated with a high mortality rate. Patients with HLH typically present with fevers, anemia/thrombocytopenia, very high serum ferritin levels, and liver abnormalities. Diagnosis requires meeting 5 of 8 diagnostic criteria. However, a presumptive diagnosis can be made and empiric therapy started without meeting the diagnostic criteria due to the life-threatening nature of HLH if there is a high clinical suspicion. Our patient initially met 4 of the criteria when empiric therapy was started, but later met a 5th. A proposed mechanism of post-partum HLH is that during pregnancy there is physiological T-cell immunomodulation and suppression of NK cell activity. This state may persist postpartum and may lead to susceptibility to HLH. There are few case reports of post-partum HLH, and most report diagnosis within the first few days after delivery. However, as it normally takes 6-8 weeks for the maternal physiology to return to the pre-pregnancy state, our patient may have been within this window of susceptibility.
Conclusions: Our case highlights the importance of correctly identifying and managing anemia in a post-partum female. Anemia in this period may be multi-factorial, and the differential includes common as well as rare conditions. HLH is one of these rare but potentially life-threatening conditions. It is therefore critical that hospitalists are aware of the signs and symptoms so that early hematology consultation can be obtained and empiric therapy started.