Familial Haemophagocytic Reticulosis

Farquhar, James W.; Macgregor, Agnes R.; Richmond, John

doi:10.1136/bmj.2.5112.1561

Cited by 98 publications

(49 citation statements)

References 1 publication

(1 reference statement)

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“…There are two main types of HLH: hereditary (familial) [2], inherited in an autosomal recessive pattern; and a secondary type which has been associated with infection, especially EBV [3]. This condition, also known as hemophagocytic syndrome, was first reported in 1939 [4].…”

Section: Discussionmentioning

confidence: 99%

Sickle cell crisis associated with hemophagocytic lymphohistiocytosis

et al. 2004

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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.

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Section: Discussionmentioning

confidence: 99%

Sickle cell crisis associated with hemophagocytic lymphohistiocytosis

et al. 2004

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“…Patients may have a variety of skin manifestations, including generalized maculopapular erythematous rashes, generalized erythroderma, edema, panniculitis, morbilliform erythema, petechiae, and purpura. 5,39 The incidence of skin manifestations ranges from 6%-65% with highly pleomorphic presentations. 4,40,7 Some patients may present with features suggestive of Kawasaki disease, including erythematous rashes, conjunctivitis, red lips, and enlarged cervical lymph nodes.…”

Section: Discussionmentioning

confidence: 99%

“…The primary autosomal recessive form, also known as familial haemophagocytic lymphohistiocytosis (FHLH), is usually seen among children though adult cases have been reported. 4 FHLH were first reported among in two siblings in 1952 5 and has an estimated incidence of 1 case per 50,000 liveborn children. 6 This variety is a fatal disease with a median survival less than 2 months after diagnosis if untreated, and it typically has its onset during infancy or early childhood.…”

Section: Introductionmentioning

confidence: 99%

Haemophagocytic lymphohistiocytosis in Adult- A Case Report and Literature Review

Hossain

Qureshi

Mahmud

et al. 2016

J. Bangladesh Coll. Phys.

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“…The term HMR was later succeeded by the disease entity known as malignant histiocytosis (MH) (Rappaport, 1966). The familial form of HLH (named FHL or FHLH) was first described in a family with two affected siblings (Farquhar & Claireaux, 1952). Risdall et al later reported a series of 19 patients with active viral infection, whose bone marrow smears disclosed histiocytic hyperplasia with prominent hemophagocytosis (Risdall et al, 1979).…”

Section: Historical Background and Terminology Of Hlhmentioning

confidence: 99%