Tuberculosis-Associated Haemophagocytic Syndrome: A Report of Two Cases and a Review of the Literature

Abstract

The haemophagocytic syndrome (HS) is an uncommon reactive proliferation of mature histiocytes, and is more frequently but not exclusively associated with infections in individuals with pre-existing immunologic abnormalities. As far as we know, only 13 cases of tuberculosis-associated HS have previously been reported. We present here two cases of disseminated tuberculosis-associated HS. Both of the cases recovered with antituberculosis therapy. High-dose methylprednisolone and intravenous immunoglobulin were added in one case because of the extremely severe clinical presentation. This therapy seemed to contribute to the favourable outcome of the patient. The similarities in HLA phenotypes of this patient and others reported in the literature may provide evidence for an underlying immune dysregulation in some cases of infection-associated HS.

Infection-associated haemophagocytosis: the tropical spectrum

“…The associations of HP with Mycobacterium tuberculosis and leishmaniasis have been observed earlier (Campo et al. , 1986; Undar et al. , 1996; Gagnaire et al.…”

“…(1979), a host of infectious agents have been implicated in the infection‐associated HPS (Campo et al. , 1986; Undar et al. , 1996; Tiab et al.…”

Immunohistochemistry in bone marrow pathology: a useful adjunct for morphologic diagnosis

“…In these cases, CD20 or CD5 are the most informative markers. The simultaneous expression of CD5 and CD20 helps to narrow down the differential diagnosis to mainly B-CLL and MCL, since both lymphomas express CD5 in more than 90% of cases [22,45,145,157]. The choice of further antibodies depends on the main differential diagnoses and the need for additional prognostic information.…”

“…Immunohistochemically, CD20 and CD79a are strongly positive, with an occasionally observed coexpression of CD43 in 20% of the cases [3,92]. Immunoglobulin heavy (typically IgM, sometimes IgG, and rarely IgA) and light chains are virtually always demonstrable as cytoplasmic staining in paraffin sections [22,45,69,70,145,157]. By definition, CD5 is negative.…”

Haemophagocytic syndrome and histiocytic necrotising lymphadenitis (Kikuchi's disease)

“…This condition has been described in the context of an autosomal recessive familial syndrome (FHS), 2 in association with viral (VAHS) 3 and other infections, 4 and in various malignant diseases, mainly of lymphoid origin. 5 The organisms linked to haemophagocytic syndrome thus far include parvovirus B19, herpes simplex virus (HSV), varicella zoster virus (VZV), Epstein-Barr virus (EBV), cytomegalovirus (CMV), influenza virus, coxsackie viruses, adenoviruses, fungi, parasites, rickettsia, Mycobacterium tuberculosis, and other bacteria, including Streptococcus pneumoniae.…”