Keeping it in the family: Three relatives with HbSC disease and simultaneous acute pulmonary emboli

Unusual cutaneous lesions indicating fat embolism syndrome in homozygous sickle cell disease

“…Of note patients with SCD may develop papules and plaques related to thrombosis due to sickled red blood cells . In several cases, bone marrow necrosis has been reported to be associated with parvovirus B19 infection as in our patient . Because of poor prognosis of fat embolism syndrome occurring when aplasia and multiorgan failure have developed, early diagnosis and treatment—relying on exchange transfusion—are recommended .…”

“…In several cases, bone marrow necrosis has been reported to be associated with parvovirus B19 infection as in our patient . Because of poor prognosis of fat embolism syndrome occurring when aplasia and multiorgan failure have developed, early diagnosis and treatment—relying on exchange transfusion—are recommended . The diagnosis should be suspected when leukocytosis with leukoerythroblastosis, relative reticulocytopenia, in association with signs of erythrophagocytosis (extremely elevated serum LDH and ferritin levels) are found.…”

“…In contrast, fat embolism syndrome is a rare but life‐threatening complication in SCD, possibly responsible for multiorgan failure . In the case described here, the clinical presentation was surprising, since classical skin lesions associated with fat embolism syndrome were until now described as petechial papules or plaques . Of note patients with SCD may develop papules and plaques related to thrombosis due to sickled red blood cells .…”

“…Fat embolism originating from focal bone marrow necrosis is frequently suspected to participate in ACS pathogenesis . In contrast, fat embolism syndrome is a rare but life‐threatening complication in SCD, possibly responsible for multiorgan failure . In the case described here, the clinical presentation was surprising, since classical skin lesions associated with fat embolism syndrome were until now described as petechial papules or plaques .…”