Abstract
The hematological manifestations of systemic lupus erythematous (SLE) are causes of morbidity and increased risk of mortality. Young patient, female, with SLE and antiphospholipid syndrome (APS) had severe pancytopenia after urinary tract infection. A biopsy of the bone marrow (BM) showed necrosis and fibrosis. The most common pathophysiological mechanism for pancytopenia is the production of peripheral antibodies. However, pancytopenia with BM aplasia or necrosis is rare. BM necrosis is more common with neoplastic diseases, severe infections or sickle cell anemia but is also reported for patients with SLE. It is seen more rarely in patients with primary APS. Changes in the BM microcirculation lead to ischemia and subsequent necrosis. The main complications are pancytopenia and embolism. BM necrosis has been appointed in few clinical studies as a possible cause for pancytopenia in SLE patients. Among the findings, BM necrosis was present in 19% of the patients. BM necrosis is a relatively rare and poor prognosis entity.
Highlights
The hematological manifestations of systemic lupus erythematous (SLE) are causes of morbidity and increased risk of mortality
The most common pathophysiological mechanism for pancytopenia is the production of peripheral antibodies
bone marrow (BM) necrosis has been appointed in few clinical studies as a possible cause for pancytopenia in SLE patients
Summary
O caso se refere a uma paciente do sexo feminino, 28 anos, branca, casada, do lar, natural e procedente de Itapetininga. A paciente foi encaminhada para o hospital de referência com pancitopenia e febre alta, apresentando diagnóstico de LES há 14 anos. A paciente foi tratada com difosfato de cloroquina 250 mg e prednisona em doses baixas (entre 5 e 20 mg). A jovem afirmou que havia cerca de nove anos que apresentara três episódios de trombose, sendo a primeira no membro inferior esquerdo, dois meses depois no membro inferior direito, e após seis meses no braço direito. Há cerca de sete anos, ela desenvolveu um episódio de pancreatite atribuída ao LES. A paciente relatou antecedente diagnóstico e tratamento de tuberculose renal havia oito anos. Cinco meses antes, ela fora diagnosticada com calculose renal sintomática, tendo sido realizada retirada cirúrgica a laser dos cálculos. A paciente foi internada e, após biópsia, foi diagnosticada com necrose de medula óssea. Teve melhora na série leucocitária, mantendo quadros de anemia e leucopenia significativos
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