HAD AN UNEVENTFUL DELIVERY? WAIT, ITS NOT OVER YET..

Case Presentation: Young female with history of juvenile rheumatoid arthritis, not on any medications, <2 weeks post-partum presented to us for the evaluation of thrombotic microangiopathy (TMA). Pregnancy was complicated by pre-eclampsia and gestational diabetes which had both improved after the birth of a heathy baby at term via an uncomplicated C-section. Patient had presented for her usual post-natal care visit where she complained of being uncharacteristically dizzy. She was referred to the ER where labs showed hemoglobin 5.8 g/dL (range 11.5-16), platelets 32 x 10^3/uL (140-440 x 10^3), creatinine 3.5 mg/dL (0.6-1.1), total bilirubin 4.0 mg/dL (0.3-1.2), direct bilirubin 1.5 mg/dL (0-0.5), AST 75 U/L (5-34), ALT 35 U/L (normal

Discussion: Pregnancy associated aHUS (P-aHUS) is a rare but life-threatening form of complement mediated TMA, with an incidence of 1 in 25,000 pregnancies (1). Despite its rarity, it accounts of 20% of all the aHUS cases in women. Majority of the cases are seen in post-partum period (2), and most of the patients end up under the care of Medicine. TTP/HUS carry a significant number of similarities to other pregnancy associated TMAs including pre-eclampsia and HELPP syndrome. It is also incredibly challenging to differentiate between TTP and aHUS themselves at the onset of lab abnormalities, as the diagnostic test (ADAMTS13 activity) takes a few days to result. aHUS carries a mortality rate of 25% in the acute phase and of those who survive, 50-60% end up with irreversible renal/neurological damage. Given the significant mortality rate, treatment is started empirically with high dose steroids and PEX. Initiation of eculizumab has been associated with improved renal outcomes and should be initiated as early as possible.

Conclusions: Internists should be cognizant of this rare life-threatening disorder, especially in patients in who are in their post-partum period. Hematology team should be consulted urgently for evaluation and patient should be transferred to a tertiary care center for initiation of PEX and eculizumab, and specialized care.