Sickle cell crisis associated with hemophagocytic lymphohistiocytosis

Abstract

Sickle-beta + (b + ) thalassemia is a double heterozygous genetic disorder characterized by both a qualitative and quantitative abnormality. We present a case of an African American male who was first diagnosed with sickle cell disease (SCD) at the age 23 years when he presented with generalized bone pain, fever, and hepatosplenomegaly. Laboratory findings included thrombocytopenia, microcytic anemia, and markedly elevated ferritin. He was subsequently diagnosed with a sickle-beta thalassemia hemoglobinopathy. Findings in the bone marrow aspirate and biopsy were consistent with hemophagocytic lymphohistiocytosis (HLH). HLH resolved with the resolution of sickle cell bone pain crisis without use of immunosuppressive therapy. To the best of our knowledge this is the first documented case of HLH associated with sickle cell bone pain crisis. Am.

Cytophagic histiocytic panniculitis associated with HBe hemoglobinopathy in a patient with hemophagocytic syndrome

“…During the course of investigations, on electrophoresis she was found to have HbE hemoglobinopathy. In literature HPS is described to be associated with sickle cell anemias [5]. To best of our knowledge this is the first case of HPS associated with HbE hemoglobinopathy.…”

“…In literature, few cases of HPS associated with sickle cell hemoglobinopathy are described. Kio E et al [5] have hypothesized that hypercytokinemia and impaired NK cell activity induced by zinc deficiency seen in sickle cell anemias might be responsible for it. Hb E is one of the world's most common and important mutations caused by substitution of glutamic acid by lysine at codon 26 of β globin gene.…”

A Case of Parvovirus-Related Haemophagocytic Lymphohistiocytosis in a Patient with HbH Disease

“…Kio et al described a case of sickle cell crisis associated with HLH [5]. The patient recovered without immunosuppression [5].…”

“…Kio et al described a case of sickle cell crisis associated with HLH [5]. The patient recovered without immunosuppression [5]. They mentioned that some cytokines implicated in HLH like IL-6, IL-2R, and TNF-alpha are raised in sickle cell disease [5].…”

Disseminated Histoplasmosis and Secondary Hemophagocytic Syndrome in a Non-HIV Patient

“…There have been 18 reported cases of disseminated histoplasmosis with the hemophagocytic syndrome [ 4 ]. Twelve of them occurred in HIV-infected patients and another case involved the development of HLH associated with a vasoocclusive crisis in a patient with hemoglobin Sb+ thalassemia [ 17 ]. Infection-associated hemophagocytic syndrome can have a poor outcome.…”