Background: Pancytopenia is a feature of many life-threateningconditions, ranging from drug-induced bone marrow hypoplasia,infection disease to severe hematologic disorders.Clinical case: 38-year-old woman was referred to the clinic forfever associated with epigastric pain and vomiting. She had ahistory of three months intermittent fever. In the Emergency de-partment: pancytopenia, impaired hepatic function and increaseof D-dimer. In our clinic at subsequent laboratory tests: hapto-globin consumed, hyperferritinemia, hypertriglyceridemia, hy-pofibrinogenemia, high IL6 values, beta2-microglobulin,EBV-DNA positivity, peripheral blood lymphocyte typing sugges-tive for chronic lymphoproliferative disorder T. Total body CTshowed bilateral pleural effusion, diffuse lymphadenopathy,marked hepato-splenomegaly. It was decided to perform BME(aspects of hemophagocytosis and peripheral T-cell lymphomanos) and to start immediately supportive and HLH specific ther-apy. During the 5th night, progressive respiratory failure so shewas intubated and transferred to the ICU, where she died theday after.Conclusions: Hemophagocytic lymphoistiocytosis (HLH) is a rarecondition characterized by a poor prognosis. Most common causesof secondary HLH are malignancies. As no single clinical manifes-tation is diagnostic for HLH, the diagnosis require high index ofsuspicion. The early recognition and treatment of this clinical con-dition are essential elements in the management of this pathology,although burdened by a high mortality rate
F. Riccomi, V.Z. (2018). A catastrophic case of fever of unknown origin. Pagepress [10.4081/itjm.2018.s2].
A catastrophic case of fever of unknown origin
L. FalsettiWriting – Review & Editing
;
2018
Abstract
Background: Pancytopenia is a feature of many life-threateningconditions, ranging from drug-induced bone marrow hypoplasia,infection disease to severe hematologic disorders.Clinical case: 38-year-old woman was referred to the clinic forfever associated with epigastric pain and vomiting. She had ahistory of three months intermittent fever. In the Emergency de-partment: pancytopenia, impaired hepatic function and increaseof D-dimer. In our clinic at subsequent laboratory tests: hapto-globin consumed, hyperferritinemia, hypertriglyceridemia, hy-pofibrinogenemia, high IL6 values, beta2-microglobulin,EBV-DNA positivity, peripheral blood lymphocyte typing sugges-tive for chronic lymphoproliferative disorder T. Total body CTshowed bilateral pleural effusion, diffuse lymphadenopathy,marked hepato-splenomegaly. It was decided to perform BME(aspects of hemophagocytosis and peripheral T-cell lymphomanos) and to start immediately supportive and HLH specific ther-apy. During the 5th night, progressive respiratory failure so shewas intubated and transferred to the ICU, where she died theday after.Conclusions: Hemophagocytic lymphoistiocytosis (HLH) is a rarecondition characterized by a poor prognosis. Most common causesof secondary HLH are malignancies. As no single clinical manifes-tation is diagnostic for HLH, the diagnosis require high index ofsuspicion. The early recognition and treatment of this clinical con-dition are essential elements in the management of this pathology,although burdened by a high mortality rateI documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.