Hemophagocytic syndrome: pitfalls in its diagnosis

Schettert, Iandara; Cardinalli, IA; Ozello, Margarete C.; Vassallo, José; Lorand-Metze, Irene; Souza, Cármino Antônio de

doi:10.1590/s1516-31801997000500007

Cited by 5 publications

(6 citation statements)

References 11 publications

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“…Hypertriglyceridemia 24,33,34 and hyperferritinemia 1,29 are commonly found in HLH. The hypertriglyceridemia occurs probably due to increased lipolytic activity induced by cytokines such as IL-1 and TNF.…”

Section: Resultsmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis in Autopsied Adults: Clinical, Laboratory and Immunohistochemical Evaluation for CD68 and CD57. Case Report and Literature Review

et al. 2019

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Hemophagocytic lymphohistiocytosis (HLH) is a rare, usually fatal and underdiagnosed autoimmune-activated disease. The present study aimed to perform a macroscopic, histopathological and immunohistochemical evaluation for CD68 and CD57 in organs of autopsied adults with HLH. A total of 604 autopsy reports were analyzed, and all the patients that filled the diagnostic criteria for HLH (n = 2) were selected. These patients were 18 and 37 years old. Were evaluated both clinical and autopsy reports and performed histopathological and immunohistochemical analysis of the liver and spleen. Both patients filled the diagnostic criteria for HLH, as well as presented common signs and symptoms of this disease, such as chills, abdominal pain, diaphoresis, and jaundice. Hemophagocytosis was observed in the spleen, bone marrow, and lymph nodes of the two patients at autopsy. Immunostaining in the liver and spleen of both patients was mainly severe for CD68, and predominantly mild for CD57, indicating a decrease in NKC numbers and an increase in the number of macrophages, respectively. This was the first study to evaluate CD57 and CD68 in autopsies of adults with HLH. Thus, more studies are required, not only to better elucidate the pathogenetic mechanisms involved in the secondary HLH, but also to disseminate the results in the clinical environment, contributing to the early diagnosis and treatment with consequent reduction of mortality rate. Keywords: Autoimmune Diseases. Histiocytosis. Biomarkers. ResumoA Linfohistiocitose Hemofagocítica (HLH) é uma doença autoimune rara, geralmente fatal e subdiagnosticada. Este estudo tem como objetivo realizar avaliação macroscópica, histopatológica e imunohistoquímica para CD68 e CD57 em órgãos de pacientes adultos com HLH submetidos a autópsia. Um total de 604 laudos de autópsias foram analisados e todos os pacientes que preencheram os critérios diagnósticos para HLH (n = 2) foram selecionados. Esses pacientes tinham 18 e 37 anos de idade. Foram analisados tanto os prontuários quanto os laudos de autópsia, bem como foram realizadas análises histopatológicas e imunohistoquímicas do fígado e baço dos pacientes. Ambos preencheram os critérios diagnósticos para HLH e apresentarem sinais e sintomas comuns da doença, como calafrios, dor abdominal, sudorese e icterícia. A hemofagocitose foi observada no baço, medula óssea e linfonodos dos dois pacientes na autópsia. A imunohistoquímica do fígado e do baço de ambos os pacientes demonstrou imunomarcação acentuada para CD68 e predominantemente discreta para CD57, que indicam diminuição do número de NKC e aumento do número de macrófagos, respectivamente. Este foi o primeiro estudo a avaliar o CD57 e CD68 em autópsias de adultos com HLH. Assim, mais estudos são necessários, não apenas para melhor elucidar os mecanismos patogenéticos envolvidos na HLH secundária, mas também para disseminar os resultados no ambiente clínico, contribuindo para o diagnóstico e tratamento precoces com consequente redução da taxa de mortalidade. Palavras-chave: Doenças Autoimunes. Histiocitose. Biomarcadores.

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Section: Resultsmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis in Autopsied Adults: Clinical, Laboratory and Immunohistochemical Evaluation for CD68 and CD57. Case Report and Literature Review

et al. 2019

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“…The sequestration of iron within the secondary lysosomal apparatus of macrophages due to erythrophagocytosis leads to the development of a capacity for iron exocytosis. There is increased production and exocytosis of free iron of released haemoglobin, reduced tissue clearance, an increased presence of interleukin (IL)‐1β, C‐reactive protein, IL‐6, tumour necrosis factor (TNF) and interferon (IFN)‐γ and hyperferritinemia 49,50 . Accordingly, the viral attack to RBCs and temporary erythrophagocytosis cause less and less haemoglobin that can carry oxygen and carbon dioxide, as well as, more and more free iron exocytosis from overloaded phagocytes.…”

Section: Receptor Recognition Mechanisms Of Coronavirusesmentioning

confidence: 99%

Targeting the glycans: A paradigm for host‐targeted and COVID‐19 drug design

Pourrajab

2021

J Cellular Molecular Medi

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There is always a need for new approaches for the control of virus burdens caused by seasonal outbreaks, the emergence of novel viruses with pandemic potential and the development of resistance to current antiviral drugs. The outbreak of the 2019 novel coronavirus‐disease COVID‐19 represented a pandemic threat and declared a public health emergency of international concern. Herein, the role of glycans for the development of new drugs or vaccines, as a host‐targeted approach, is discussed where may provide a front‐line prophylactic or threats to protect against the current and any future respiratory‐infecting virus and possibly against other respiratory pathogens. As a prototype, the role of glycans in the coronavirus infection, as well as, galectins (Gal) as the glycan‐recognition agents (GRAs) in drug design are here summarized. Galectins, in particular, Gal‐1 and Gal‐3 are ubiquitous and important to biological systems, whose interactions with viral glycans modulate host immunity and homeostatic balance.

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“…Both HLH and disseminated NTM infections can be fatal, prompt diagnosis is mandatory for improving patient's outcome, especially those are endogenously or therapeutically immunosuppressed (Shi and Jiao 2019). Of note, there is currently no documented relationship between MP and HLH, although HLH can develop in association with panniculitis character-istic of Weber-Christian disease (WCD) (Schettert et al 1997). WCD is a systemic inflammatory disease accompanied by relapsing panniculitis, fever, lipoatrophy, and lipophagia (White and Winkelmann 1998), which in part resembles with MP manifestations.…”

Section: Introductionmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis Associated with Disseminated Nontuberculous Mycobacterial Infection in a Patient with Mesenteric Panniculitis

Fujita

Matsumoto

Asano

et al. 2021

Tohoku J. Exp. Med.

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Mesenteric panniculitis is a chronic inflammatory disease characterized by non-specific inflammation of the adipose tissue in the mesentery. Hemophagocytic lymphohistiocytosis is a life-threating disease associated with aberrant macrophage overactivation, in which infections can be a leading cause in immunocompromised hosts. Here, we report a rare case of mesenteric panniculitis and hemophagocytic lymphohistiocytosis complicated by disseminated Mycobacterium intracellulare. A 71-year-old male with mesenteric panniculitis was admitted to our hospital for fever and pancytopenia. He was treated with oral prednisolone (15 mg/day) and cyclosporin A (150 mg/day) at presentation. Physical and laboratory examinations revealed disseminated infection with nontuberculous mycobacteria; Mycobacterium intracellulare was detected in cultures of cerebrospinal fluid, blood, sputum, and gastric fluid. Patient signs and symptoms fulfilled the five criteria for a diagnosis of hemophagocytic lymphohistiocytosis, including fever, cytopenia, hemophagocytosis, hyperferritinemia, and high soluble interleukin-2 receptor levels. Therefore, the diagnosis of nontuberculous mycobacteria-associated hemophagocytic lymphohistiocytosis was established. An anti-mycobacterial chemotherapy including chloramphenicol (800 mg/day), rifampin (450 mg/day) and ethambutol (750 mg/day) together with streptomycin (750 mg twice per week) was initiated at 30 days after admission; maintenance doses of prednisolone were increased to 60 mg/day. Fever and pancytopenia improved in response to anti-mycobacterial chemotherapy. The present case suggests that mesenteric panniculitis could be complicated with hemophagocytic lymphohistiocytosis caused by immunosuppressive therapy-associated infections as well as underlying disease activity. In conclusion, the possibility of disseminated nontuberculous mycobacteria infection with hemophagocytic lymphohistiocytosis should be considered if unexplained fever or hematological dyscrasia were presented in patients of mesenteric panniculitis.

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Hemophagocytic syndrome: pitfalls in its diagnosis

Cited by 5 publications

References 11 publications

Hemophagocytic Lymphohistiocytosis in Autopsied Adults: Clinical, Laboratory and Immunohistochemical Evaluation for CD68 and CD57. Case Report and Literature Review

Hemophagocytic Lymphohistiocytosis in Autopsied Adults: Clinical, Laboratory and Immunohistochemical Evaluation for CD68 and CD57. Case Report and Literature Review

Targeting the glycans: A paradigm for host‐targeted and COVID‐19 drug design

Hemophagocytic Lymphohistiocytosis Associated with Disseminated Nontuberculous Mycobacterial Infection in a Patient with Mesenteric Panniculitis

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