Development of hemophagocytic lymphohistiocytosis in triplets infected with HHV-8

Grossman, William; Radhi, Mohammed; Schauer, Dennis W.; Gerday, Erick; Grose, Charles; Goldman, Frederick D.

doi:10.1182/blood-2005-03-0950

Cited by 40 publications

(28 citation statements)

References 18 publications

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“…9,10 Viral infections may elicit the onset of disease. 11,12 Specific criteria for diagnosis of HLH have been established and were recently revised. 13 Typical findings include fever, massive hepatosplenomegaly, cytopenia, and hyperferritinemia.…”

Section: Introductionmentioning

confidence: 99%

Defective cytotoxic lymphocyte degranulation in syntaxin-11–deficient familial hemophagocytic lymphohistiocytosis 4 (FHL4) patients

Bryceson

Rudd

Zheng

et al. 2007

Blood

276

314

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Section: Introductionmentioning

confidence: 99%

Defective cytotoxic lymphocyte degranulation in syntaxin-11–deficient familial hemophagocytic lymphohistiocytosis 4 (FHL4) patients

Bryceson

Rudd

Zheng

et al. 2007

Blood

276

314

View full text Add to dashboard Cite

show abstract

“…Case studies have described this complication in 2 of 3 triplet infants without HIV infection [88], in HIV-positive patients with known KS [89], and in a 2.5-year-old boy with X-linked lymphoproliferative phenotype [90]. Patients with primary immune deficiency may also be at risk for disseminated HHV-8 infection, as has been reported with a fatal outcome in a young patient with DiGeorge syndrome [91].…”

Section: Other Pediatric Manifestationsmentioning

confidence: 85%

A Review of Human Herpesvirus 8, the Kaposi's Sarcoma-Associated Herpesvirus, in the Pediatric Population

Dow

Cunningham

Buchanan

2013

Journal of the Pediatric Infectious Diseases Society

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“…Although concurrent infectious diseases were excluded, HHV8 leading to Castleman disease and Kaposi sarcoma are established but rare causes of HLH. [77][78][79] Epstein-Barr virus (EBV) is also a wellknown infectious cause of HLH. Although the contribution of EBV to the development of HLH is beyond the scope of this article, it is notable that EBV-driven lymphomas contributed significantly to the results.…”

Section: Diagnosis and Prognosismentioning

confidence: 99%

Proliferation through activation: hemophagocytic lymphohistiocytosis in hematologic malignancy

Vick¹,

Patel

Prouet

et al. 2017

Blood Advances

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Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of cytokine-driven immune activation. Cardinal features include fever, hemophagocytosis, hepatosplenomegaly, lymphocytic infiltration, and hypercytokinemia that result in multisystem organ dysfunction and failure. Familial HLH is genetically driven, whereas secondary HLH (SHL) is caused by drugs, autoimmune disease, infection, or cancer. SHL is associated with worse outcomes, with a median overall survival typically of less than 1 year. This reflects difficulty in both diagnostic accuracy and in establishing reliable treatments, especially in cases of malignancyinduced SHL, which have significantly worse outcomes. Malignancy-induced HLH is seen almost exclusively with hematologic malignancies, constituting 97% of cases in the literature over the past 2 years. In these situations, the native immune response driven by CD8 T cells produces an overabundance of T helper 1 cytokines, notably interferon-g, tumor necrosis factor-a, and interleukin-6, which establish a positive feedback loop of inflammation, enhancing replication of hematologic malignancies while leaving the host immune system in disarray. In this paper, we present 2 case studies of secondary HLH driven by HM, followed by a review of the literature discussing the cytokines driving HLH, diagnostic criteria, and current treatments used or undergoing investigation. Case 1A previously healthy 34-year-old male presented with progressive malaise, fevers, and abdominal discomfort. He was found to have massive splenomegaly along with pancytopenia and coagulopathy. Initial laboratory studies showed lactate dehydrogenase (LDH) 470 U/L, ferritin 4450 ng/dL, white blood cell count 1.3 3 10 9 /L, platelets 31 3 10 9 /L, hematocrit 22%, and fibrinogen level 130 mg/dL. A bone marrow (BM) biopsy was performed and showed lymphohistiocytic aggregation without hemophagocytosis. The patient underwent splenectomy; pathology showed splenic red pulp congestion and proliferation of sheets of normal histiocytes with marked erythrophagocytosis. No conclusive evidence of B-or T-cell lymphoma was found at that time, although features suggestive of but not diagnostic for T-cell rich diffuse large B-cell lymphoma (DLBCL) were seen in the spleen. The patient subsequently had a positron emission tomography/computed tomography scan and was found to have small hyperactive para-aortic lymph nodes that were not accessible for biopsy. The patient began therapy for HLH with dexamethasone and etoposide for 6 cycles and tolerated it well, with resolution of his laboratory abnormalities and symptoms.A year and a half after initial diagnosis, he presented to the hospital again with back pain and fevers. A computed tomography scan showed multiple retroperitoneal and periaortic lymph nodes along with liver lesions. He was started on dexamethasone and admitted to the hospital. Multiple lymph node biopsies were performed and were inconclusive, possibly from steroid pretreatment. Further evaluation with bilateral BM biopsies reported T-cell rich ...

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Development of hemophagocytic lymphohistiocytosis in triplets infected with HHV-8

Cited by 40 publications

References 18 publications

Defective cytotoxic lymphocyte degranulation in syntaxin-11–deficient familial hemophagocytic lymphohistiocytosis 4 (FHL4) patients

Defective cytotoxic lymphocyte degranulation in syntaxin-11–deficient familial hemophagocytic lymphohistiocytosis 4 (FHL4) patients

A Review of Human Herpesvirus 8, the Kaposi's Sarcoma-Associated Herpesvirus, in the Pediatric Population

Proliferation through activation: hemophagocytic lymphohistiocytosis in hematologic malignancy

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