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1.
J Med Case Rep ; 15(1): 98, 2021 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-33648567

RESUMO

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is characterized by hyperinflammation and life-threatening cytopenias. Survival is poor, and management is pivotal on rapid identification of the disease. HLH is associated with hematologic malignancies, however correlation with myelodysplastic syndromes (MDS) is exceedingly unusual. Although minimizing overwhelming hyperinflammation by treating hemophagocytosis are central for HLH outcome, there is urgent necessity to identify potential initiating mechanisms that could assist in therapy design. CASE DESCRIPTION: Here, we describe an elderly African American patient who developed rapid onset of cytopenias and coagulopathy associated with hepatic and bone marrow hemophagocytosis. We analyze four additional similar cases to isolate clinical, laboratory and cytogenetic findings expected in patients exhibiting concurrent HLH and MDS. HLH linked with MDS retains common HLH features associated with systemic hyperinflammation such as fever, hypotension, hepatosplenomegaly, hyperferritinemia, coagulopathy and rapidly evolving cytopenias. Typical MDS chromosomic abnormality such as trisomy 8 was frequently observed in our studied cases. CONCLUSION: Our case describes difficulties while managing HLH in MDS patients. Diagnosis should be based on identifying HLH appropriate criteria and if possible karyotypic abnormalities normally observed in MDS.


Assuntos
Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/genética , Síndromes Mielodisplásicas/diagnóstico , Síndromes Mielodisplásicas/genética , Idoso de 80 Anos ou mais , Medula Óssea/patologia , Cromossomos Humanos Par 8 , Evolução Fatal , Feminino , Humanos , Cariótipo , Trissomia
2.
Artigo em Inglês | MEDLINE | ID: mdl-33656138

RESUMO

Congenital cytomegalovirus infection causes lethal diseases with neurological, visual, auditory and systemic injuries, including the hemophagocytic syndrome. Hemophagocytic lymphohistiocytosis (HLH) can be caused by primary hereditary immunological defects, as well as several infectious triggering factors, such as viruses, bacteria and fungus, among them the cytomegalovirus (CMV). Here we present the case report of a male newborn male, delivered by cesarean at term (gestation age of 39 weeks), weighing 3,250 g, with suffusion skin lesions spread throughout the body, anemia, generalized edema, hepatosplenomegaly, thrombocytopenia associated with grunts and difficulty breathing, treated with ganciclovir after receiving the diagnosis of congenital CMV infection. After a few days of hospitalization, the patient presented with high fever, persistent hepatosplenomegaly and pancytopenia, in addition to elevated ferritin and triglycerides, receiving the diagnosis of HLH treated with immunosuppressive therapy, corticosteroids and intravenous human immunoglobulin. The present case report highlights the importance for health professionals to carry out the investigation of congenital diseases, especially in developing countries, as well as their complications, such as HLH.


Assuntos
Infecções por Citomegalovirus/congênito , Citomegalovirus/isolamento & purificação , Linfo-Histiocitose Hemofagocítica/diagnóstico , Administração Intravenosa , Corticosteroides/administração & dosagem , Cesárea , Infecções por Citomegalovirus/diagnóstico , Infecções por Citomegalovirus/tratamento farmacológico , Feminino , Humanos , Imunoglobulinas/administração & dosagem , Lactente , Recém-Nascido , Linfo-Histiocitose Hemofagocítica/complicações , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Masculino , Gravidez , Complicações Infecciosas na Gravidez/diagnóstico , Complicações Infecciosas na Gravidez/tratamento farmacológico , Resultado do Tratamento
5.
Medicine (Baltimore) ; 100(12): e25170, 2021 Mar 26.
Artigo em Inglês | MEDLINE | ID: mdl-33761694

RESUMO

RATIONALE: The immunologic syndrome induced by severe acute coronavirus disease 2019 (COVID-19) is yet not fully understood. Typical patterns of clinical and laboratory features match secondary hemophagocytic lymphohistiocytosis (sHLH). However, the optimal approach to COVID-19 patients testing positive for sHLH is still unclear. PATIENT CONCERNS: Three patients with COVID-19 are reviewed. All showed hyperinflammation and cytokine storm, necessitating intensive care treatment including mechanical ventilation. DIAGNOSIS: Secondary hemophagocytic lymphohistiocytosis due to severe COVID-19; diagnosed via HScore. INTERVENTIONS: A treatment regimen of methylprednisolone, pentaglobin, and anakinra was developed and administered. OUTCOMES: One patient survived the ICU stay. Two other patients, in whom sHLH was diagnosed too late, deceased. LESSONS: A routine screening of COVID-19 patients for secondary HLH by using the HScore is feasible; especially those patients deteriorating clinically with no sufficient response to shock management might be at particular high risk. A stepwise therapeutic approach comprising corticosteroids, immunoglobulins and anakinra, accompanied by immunoadsorption, may dampen cytokine storm effects, and potentially reduce mortality.


Assuntos
/complicações , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Linfo-Histiocitose Hemofagocítica/etiologia , Idoso , Anti-Inflamatórios/uso terapêutico , /terapia , Terapia Combinada , Cuidados Críticos , Síndrome da Liberação de Citocina/tratamento farmacológico , Diagnóstico Tardio , Evolução Fatal , Feminino , Humanos , Imunoglobulina A/uso terapêutico , Imunoglobulina M/uso terapêutico , Imunoglobulinas Intravenosas/uso terapêutico , Imunossupressores/uso terapêutico , Proteína Antagonista do Receptor de Interleucina 1/uso terapêutico , Linfo-Histiocitose Hemofagocítica/diagnóstico , Masculino , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Respiração Artificial
7.
Yonsei Med J ; 62(3): 274-277, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33635018

RESUMO

Hemophagocytic syndrome (HPS) is a rare but potentially life-threatening disease in kidney transplant recipients, and is caused by systemic proliferation of macrophages actively phagocytizing other blood cells in the bone marrow, lymph nodes, and the spleen. Here, we report a 40-year-old male kidney transplant recipient who presented with fever, bicytopenia, and elevated liver enzymes 2 months after transplantation. Given that cytomegalovirus antigenemia and real-time polymerase chain reaction tests were positive, liver biopsy was performed under an assumption of cytomegalovirus-induced hepatitis. Hepatic histology revealed multifocal microabscess with cytomegalovirus inclusion bodies, marked Kupffer cell hyperplasia, and erythrophagocytosis by activated macrophages. As laboratory findings such as hyperferritinemia, elevated serum lactate dehydrogenase, low natural killer cell activity, and high soluble interleukin-2 receptor were also compatible with HPS, the recipient was diagnosed as having cytomegalovirus-induced hepatitis combined with reactive HPS. Following intravenous ganciclovir therapy with continuous administration of tacrolimus and corticosteroid, the symptoms resolved and laboratory findings were normalized. As far as we know, this is the first report of cytomegalovirus-induced hepatitis combined with reactive HPS in a kidney transplant recipient that is diagnosed by liver biopsy.


Assuntos
Infecções por Citomegalovirus/virologia , Citomegalovirus/fisiologia , Transplante de Rim/efeitos adversos , Fígado/patologia , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/virologia , Adulto , Antivirais/uso terapêutico , Biópsia , Humanos , Linfo-Histiocitose Hemofagocítica/diagnóstico por imagem , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Masculino , Tacrolimo/uso terapêutico , Tomografia Computadorizada por Raios X
8.
Zhongguo Shi Yan Xue Ye Xue Za Zhi ; 29(1): 253-258, 2021 Feb.
Artigo em Chinês | MEDLINE | ID: mdl-33554830

RESUMO

OBJECTIVE: To present one patient initially diagnosed with dermatomyositis(DM) who was eventually revealed to be diffuse large B-cell lymphoma(DLBCL) complicated with hemophagocytic syndrome(HPS), and to improve the understanding of the disease. METHODS: The clinical characteristics, diagnostic approach, treatment of the patient were retrospectively analyzed, and some related literatures were reviewed. RESULTS: A 52-year-old female patient suffered from muscle weakness, elevated serum creatine kinase activity, electromyography changes and characteristic skin rashes and diagnosed as DM. The patient was treated with glucocorticoid therapy and the muscle strength, skin rashes, and creatine kinas index turns into remission. Subsequently, subcutaneous nodules appeared during treatment, and the patient was confirmed as DLBCL based on pathological biopsy; And the patient was considered HPS because of presenting with repeated fever, splenomegaly, cytopenias, hypofibrinogenemia, hypertriglyceridemia, hyperferritinemia, high levels of sCD25, low NK-cell activity and hemophagocytosis in bone marrow. But the patient refused chemotherapy, and only treated with "DXM+VP-16" to control hemophagocytic syndrome, and unfortunately died due to the disease progression. CONCLUSION: Cutaneous involvement in diffuse large B-cell lymphoma and hemophagocytic syndrome patients with first presentation of dermatomyositis is relatively rare. Malignacy screening should be performed as soon as possible after newly diagnosed DM, so that the patient can get early diagnosis and effective treatment to improve survival rate.


Assuntos
Dermatomiosite , Linfo-Histiocitose Hemofagocítica , Linfoma Difuso de Grandes Células B , Dermatomiosite/complicações , Etoposídeo , Feminino , Humanos , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfoma Difuso de Grandes Células B/complicações , Pessoa de Meia-Idade , Estudos Retrospectivos
9.
Med Klin Intensivmed Notfmed ; 116(2): 129-134, 2021 Mar.
Artigo em Alemão | MEDLINE | ID: mdl-33580314

RESUMO

Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammation syndrome. In adults, secondary HLH is mostly observed. HLH is often triggered by infections, malignancies or autoimmune disorders. However, HLH cases in association with immunotherapies have been described recently. HLH in critically ill patients is often difficult to differentiate from sepsis. Both conditions can also be present at the same time. Early diagnosis and timely initiation of an adequate immunosuppressive therapy are essential for the further course and the prognosis of HLH. Therefore, HLH should represent a differential diagnosis in critically ill patients with persistent fever and additional symptoms (e.g. enlarged spleen, neurologic symptoms) or laboratory parameters (e.g. hyperferritinemia, cytopenia, increased transaminases) compatible with HLH. The diagnosis of HLH is made using the HLH-2004 criteria. The probability of the presence of HLH can be calculated using the HScore. High-dose corticosteroids represent the cornerstone of HLH treatment. Etoposide, immunoglobulins, anakinra or other drugs are added depending on the trigger. The course of HLH is influenced by the time of treatment initiation, the underlying trigger and the response to treatment. Generally, the prognosis of critically ill HLH patients is poor.


Assuntos
Linfo-Histiocitose Hemofagocítica , Sepse , Adulto , Estado Terminal , Diagnóstico Diferencial , Humanos , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/terapia
11.
Clin Rheumatol ; 40(4): 1233-1244, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33389315

RESUMO

Primary and secondary haemophagocytic lymphohistiocytosis (HLH) are hyperferritinaemic hyperinflammatory syndromes with a common terminal pathway triggered by different etiopathogenetic factors. HLH is characterised by a decreased capacity of interferon gamma production with an activated NK phenotype profile similar to other hyperinflammatory syndromes. Viruses are closely linked to the development of HLH as infectious triggers, and the break of tolerance to self-antigens is considered a critical mechanism involved in the development of immune-mediated conditions triggered by viral infections. Emerging studies in patients with COVID-19 are suggesting a key role of monocytes/macrophages in the pathogenesis of this viral infection, and there is a significant overlap between several features reported in severe COVID-19 and the features included in the HLH-2004 diagnostic criteria. Therefore, SARS-Cov-2, as other respiratory viruses, may also be considered a potential etiological trigger of HLH. The frequency of HLH in adult patients with severe COVID-19 is lower than 5%, although this figure could be underestimated considering that most reported cases lacked information about some specific criteria (mainly the histopathological criteria and the measurement of NK cell function and sCD25 levels). Because HLH is a multi-organ syndrome, the diagnostic approach in a patient with severe COVID-19 in whom HLH is suspected must be carried out in a syndromic and holistic way, and not in the light of isolated clinical or laboratory features. In COVID-19 patients presenting with persistent high fever, progressive pancytopenia, and hepatosplenic involvement, together with the characteristic triad of laboratory abnormalities (hyperferritinaemia, hypertriglyceridaemia, and hypofibrinogenaemia), the suspicion of HLH is high, and the diagnostic workup must be completed with specific immunological and histopathological studies.


Assuntos
Síndrome da Liberação de Citocina/diagnóstico , Linfo-Histiocitose Hemofagocítica/diagnóstico , Síndrome de Ativação Macrofágica/diagnóstico , Adulto , /diagnóstico , Criança , Síndrome da Liberação de Citocina/etiologia , Síndrome da Liberação de Citocina/fisiopatologia , Diagnóstico Diferencial , Humanos , Linfo-Histiocitose Hemofagocítica/complicações , Linfo-Histiocitose Hemofagocítica/fisiopatologia , Síndrome de Ativação Macrofágica/fisiopatologia , Pandemias , Reumatologia/métodos
12.
BMJ Case Rep ; 14(1)2021 Jan 06.
Artigo em Inglês | MEDLINE | ID: mdl-33408106

RESUMO

Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening disorder of excessive immune activation. It is mostly seen in the paediatric population and is rarely observed in adults. HLH can be inherited or acquired and is commonly triggered by activation of the immune system by an underlying viral infection or in immune system deficiency such as malignancy or underlying rheumatological disease. HLH is a difficult entity to diagnose due to the rarity of this disorder, variable clinical presentation and non-specific clinical and laboratory findings. HLH carries a high mortality if left untreated, and therefore prompt diagnosis and initiation of immunosuppressive, immunomodulatory and cytostatic medications are critical to improve survival in affected patients. Here, we present a case of lamotrigine-associated HLH. To our knowledge, only eight other cases of lamotrigine-associated HLH have been reported in adult patients.


Assuntos
Anticonvulsivantes/administração & dosagem , Lamotrigina/efeitos adversos , Linfo-Histiocitose Hemofagocítica/induzido quimicamente , Convulsões/tratamento farmacológico , Adulto , Biópsia , Medula Óssea/patologia , Dexametasona/uso terapêutico , Diagnóstico Diferencial , Etoposídeo/uso terapêutico , Humanos , Linfo-Histiocitose Hemofagocítica/sangue , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Masculino
13.
Ital J Pediatr ; 47(1): 3, 2021 Jan 07.
Artigo em Inglês | MEDLINE | ID: mdl-33413556

RESUMO

OBJECTIVES: This study aimed to compare the clinical features and laboratory tests of infectious mononucleosis (IM) and hemophagocytic syndrome (HLH) caused by Epstein-Barr virus (EBV) in 1-3-year-old children and to explore the risk factor of HLH caused by EBV (EBV-HLH). METHODS: The clinical data of 92 children with EBV infection admitted in our hospital from 2011 to 2019 were collected; 61 cases were diagnosed as EBV-IM, and 31 cases were diagnosed as EBV-HLH. The subjects' clinical manifestations and laboratory tests were analyzed retrospectively. RESULTS: Compared with EBV-IM patients, EBV-HLH patients had longer durations of fever, both before hospitalization and overall, and a higher probability of hepatomegaly. The levels of ALT, AST, LDH, TG, SF, D-Dimer and the plasma EBV DNA load of EBV-HLH patients were significantly higher than those of EBV-IM patients. The absolute values of CD3+, CD4+, CD8+, NK, and CD3-CD19+ cells and IgA and IgM levels of EBV-HLH patients were significantly lower than those of EBV-IM patients. The plasma EBV DNA load was positively correlated with the PT, TT, α-HBDH, AST, LDH, CK, Scr, BUN, UA, TG, and CRP levels in EBV-HLH patients, and the plasma EBV DNA load was positively correlated with the D-Dimer level in the EBV-IM patients. Among the 10 different potential markers, at the cut-off point of 1721.500 µg/L, the sensitivity and specificity of D-Dimer was 88.90 and 90.20%, respectively. CONCLUSION: The D-Dimer level may be a good prognostic indicator of EBV-HLH caused by EBV.


Assuntos
Herpesvirus Humano 4 , Mononucleose Infecciosa/diagnóstico , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/virologia , Pré-Escolar , Feminino , Hospitalização , Humanos , Lactente , Mononucleose Infecciosa/complicações , Linfo-Histiocitose Hemofagocítica/complicações , Masculino , Estudos Retrospectivos , Sensibilidade e Especificidade , Avaliação de Sintomas , Carga Viral
14.
Am J Case Rep ; 22: e927142, 2021 Jan 11.
Artigo em Inglês | MEDLINE | ID: mdl-33428607

RESUMO

BACKGROUND Subcutaneous panniculitis-like T cell lymphoma and primary cutaneous γdelta T cell lymphoma are rare forms of non-Hodgkin lymphoma presenting as skin nodules or plaques. CASE REPORT Here, we present a case of a 48-year-old man with multiple subcutaneous, tender, erythematous nodules on his right thigh and left arm. Multiple courses of antibiotics were administered with no significant improvement in the patient's lesions. The skin biopsy report showed CD3/CD8 lymphocytic rimming of the adipocytes and the patient was diagnosed with subcutaneous panniculitis-like T cell lymphoma. A subsequent bone marrow biopsy showed hemophagocytic lymphohistiocytosis. The patient underwent treatment with the cyclophosphamide, hydroxydaunorubicin, Oncovin, prednisone, and etoposide chemotherapy regimen and received an autologous peripheral blood stem cell transplant. CONCLUSIONS Nodular skin lesions can result from a variety of noninfectious causes in addition to bacterial and fungal infections. This case highlights the importance of early biopsy of skin lesions that do not respond to standard therapy to establish an accurate diagnosis and start timely treatment to prevent poor outcomes.


Assuntos
Celulite (Flegmão)/diagnóstico , Linfo-Histiocitose Hemofagocítica/complicações , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfoma de Células T/complicações , Linfoma de Células T/diagnóstico , Paniculite/complicações , Paniculite/diagnóstico , Diagnóstico Diferencial , Humanos , Linfo-Histiocitose Hemofagocítica/terapia , Linfoma de Células T/terapia , Masculino , Pessoa de Meia-Idade , Paniculite/terapia
15.
Int J Infect Dis ; 103: 624-627, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33412272

RESUMO

A 21-year-old woman was hospitalized due to coronavirus disease 2019 (COVID-19)-associated respiratory and hepatic impairment concomitant with severe hemolytic anemia. Upon diagnosis of secondary hemophagocytic lymphohistiocytosis, immunosuppression with anakinra and steroids was started, leading to a hepatic severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and viremia. Subsequent liver biopsy revealed virus particles in hepatocytes by electron microscopy and SARS-CoV-2 virus could be isolated and cultured. Immunosuppression was stopped and convalescent donor plasma given. In the differential diagnosis, an acute crisis of Wilson's disease was raised by laboratory and genetic testing. This case highlights the complexity of balancing immunosuppression to control hyperinflammation versus systemic SARS-CoV-2 dissemination.


Assuntos
/complicações , Degeneração Hepatolenticular/diagnóstico , Fígado/virologia , Linfo-Histiocitose Hemofagocítica/etiologia , Diagnóstico Diferencial , Feminino , Humanos , Imunossupressão , Linfo-Histiocitose Hemofagocítica/diagnóstico , Adulto Jovem
16.
Am J Med Sci ; 361(3): 388-393, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-33309387

RESUMO

We report, to the best of our best knowledge, the oldest individual to ever be diagnosed with Familial Hemophagocytic Lymphohistiocytosis (FHL) Type 2 from homozygous c.1349C>T (p.T450M) missense variants in the PRF1 gene. This rare case advanced in complexity with a simultaneous diagnosis of Chronic Active Epstein-Barr Virus (CAEBV) - a distinct clinical entity from acute EBV infections and a well-described trigger of Hemophagocytic Lymphohistiocytosis (HLH). This is, to the best of our knowledge, the only individual to ever be diagnosed with CAEBV in the setting of this specific variant and the oldest to be diagnosed with a coexisting perforin variant. This case provides understanding of EBV, human genetics, and lymphoproliferative disorders while adding a unique differential diagnosis to adults who present with fever of unknown origin and diffuse lymphadenopathy without evidence of malignancy. This report explores the diagnosis and treatment of both HLH and CAEBV, encouraging discussion regarding current clinical management and future research needs.


Assuntos
Infecções por Vírus Epstein-Barr/diagnóstico , Linfo-Histiocitose Hemofagocítica/diagnóstico , Adulto , Doença Crônica , Diagnóstico Diferencial , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/virologia , Herpesvirus Humano 4/isolamento & purificação , Humanos , Índia/etnologia , Linfo-Histiocitose Hemofagocítica/complicações , Linfo-Histiocitose Hemofagocítica/genética , Masculino , Perforina/análise
18.
J Formos Med Assoc ; 120(1 Pt 1): 172-179, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32307323

RESUMO

PURPOSE: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease associated with rapid clinical deterioration and the need for intensive care; therefore, it is essential to identify clinical parameters related to mortality and establish prognostic factors correlated with unfavorable outcome in high risk patients whose treatment may fail. METHODS: Between January 2004 and December 2018, a total of 51 pediatric patients (less than 18 years old) who fulfilled the diagnostic criteria of HLH-2004 with documented results of bone marrow investigations at Kaohsiung Chang Gung Memorial Hospital were enrolled. The treatment protocol was based on hemophagocytic lymphohistiocytosis-94 (HLH-94) and HLH-2004. We retrospectively reviewed electronic medical records (EMR) including clinical features, length of intensive care unit (ICU) stay, serological tests, microscopic reports of bone marrow examination, and ultrasound examination reports at diagnosis to identify prognostic factors. The patients were divided into four groups based on etiology; these included infection associated hemophagocytic syndrome (IAHS), macrophage activation syndrome (MAS), malignancy associated hemophagocytic lymphohistiocytosis (MA-HLH), and idiopathic hemophagocytic lymphohistiocytosis (IHLH) to identify differences among the groups. RESULTS: Out of 51 patients enrolled, 27 patients had IAHS, 12 MAS, 8 MA-HLH, and 4 IHLH. The median age at diagnosis was 7 years. The overall mortality rate was 15.7% (there was no mortality in the MA-HLH group); the mean length of ICU stay was 6 ± 20.8 days. Longer activated partial thromboplastin time (aPTT) (p = 0.007), lower sodium concentration (p = 0.0007), and higher creatinine (p = 0.032) and aspartate aminotransferase (AST) (p = 0.017) were significantly related to mortality. Multivariate Cox regression analysis demonstrated that aPTT (p = 0.045, HR = 1.03, 95% CI = 1.0-1.1) was an independent risk factor for mortality. The receiver operating characteristic (ROC) curve showed that aPTT longer than 44.35 s was the cutoff value predicting mortality, with a sensitivity and specificity of 72% and 66.7%, respectively. CONCLUSION: MA-HLH had the lowest mortality rate, as most children died from the underlying malignant disease and not from HLH. Impaired liver and renal functions were related to mortality. Prolonged aPTT > 44.35 s is a strong predictive factor for mortality.


Assuntos
Linfo-Histiocitose Hemofagocítica , Neoplasias , Adolescente , Criança , Humanos , Unidades de Terapia Intensiva , Linfo-Histiocitose Hemofagocítica/diagnóstico , Estudos Retrospectivos
20.
Artigo em Inglês | MEDLINE | ID: mdl-33331518

RESUMO

Hemophagocytic lymphohistiocytosis is a rare complication in Rickettsia typhi infections. We report the case of a 2-year-old boy with sudden night-onset fever, pallor, neck adenopathy and erythematous macular rash on the thorax, thighs and buttocks. During admission, he developed hyponatremia, hypoalbuminemia, anemia, thrombocytopenia, leukopenia, neutropenia, liver damage, hemorrhages and persistent fever. No hematological improvement was observed after the initial management, neoplastic diseases were discarded by bone marrow aspiration and lymph node biopsy; hemophagocytic lymphohistiocytosis was diagnosed. By immunohistochemistry and indirect immunofluorescence, murine typhus was also diagnosed and doxycycline was started with transitory recovery. Later, the child developed kidney failure and distributive shock that evolved to cardiac arrest and death. This is the first case report in Mexico on a fatal murine typhus associated with hemophagocytic lymphohistiocytosis in which the etiology was evidenced by histopathology.


Assuntos
Linfo-Histiocitose Hemofagocítica/diagnóstico , Rickettsia typhi/isolamento & purificação , Tifo Endêmico Transmitido por Pulgas/diagnóstico , Animais , Medula Óssea , Pré-Escolar , Cães , Evolução Fatal , Humanos , Linfo-Histiocitose Hemofagocítica/complicações , Linfo-Histiocitose Hemofagocítica/mortalidade , México , Camundongos , Tifo Endêmico Transmitido por Pulgas/complicações , Tifo Endêmico Transmitido por Pulgas/mortalidade
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