Analysis of Triglyceride Value in the Diagnosis and Treatment Response of Secondary Hemophagocytic Syndrome

Okamoto, M; Yamaguchi, Hiroki; Isobe, Yasushi; Yokose, Norio; Mizuki, Taro; Takagi, Kenji; Gomi, Seiji; Hamaguchi, Hiroyuki; Inokuchi, Koiti; Oshimi, Kazuo; Dan, Kazuo

doi:10.2169/internalmedicine.48.1677

Cited by 48 publications

(39 citation statements)

References 28 publications

(19 reference statements)

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“…(5) The disease is classified as primary or secondary HPS. (6) The two forms are clinically indistinguishable, being characterized by a sepsis-like presentation with splenomegaly, cytopenia, hyperferritinemia, bleeding disorder, and hemophagocytosis. Consequently, multiorgan failure often develops, leading to high mortality.…”

Section: Discussionmentioning

confidence: 99%

“…(7) Primary HPS is hereditary, and most patients present with symptoms within two years after birth, being treated in the pediatric field. (6) The primary form of HPS has an autosomal recessive pattern of inheritance, with an incidence of approximately 1/50,000 live births. (7) Primary HPS can result from autosomal defects or be associated with immune deficiencies, such as Chediak-Higashi syndrome, X-linked lymphoproliferative syndrome, and Griscelli syndrome.…”

Section: Discussionmentioning

confidence: 99%

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Síndrome hemofagocítica devido a sarcoidose pulmonar

et al. 2012

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Although hemophagocytic syndrome is a rare clinical condition, it is associated with high mortality and the number of cases described in the literature has progressively increased. The diagnosis of hemophagocytic syndrome is made on the basis of a finding of hemophagocytosis. Sarcoidosis is a highly prevalent disease whose course and prognosis might correlate with the initial clinical presentation and the extent of the disease. We report the case of a patient with long-standing sarcoidosis who presented with intermittent fever and fatigue. The diagnosis of hemophagocytic syndrome was made by bone marrow aspiration, and specific treatment was ineffective. This is the third case of sarcoidosis-related hemophagocytic syndrome reported in the literature and the first reported in Latin America. All three cases had unfavorable outcomes.Keywords: Lymphohistiocytosis, hemophagocytic; Ferritins; Sarcoidosis, pulmonary; Macrophage activation syndrome. ResumoEmbora seja uma condição clínica rara, a síndrome hemofagocítica é associada com alta mortalidade e o número de casos descritos na literatura vem aumentando progressivamente. O diagnóstico de síndrome hemofagocítica depende da presença de hemofagocitose. A sarcoidose é uma doença de alta prevalência cujo curso e prognóstico podem correlacionar-se com a apresentação clínica inicial e a extensão da doença. Relatamos o caso de um paciente com sarcoidose de longa duração que apresentava febre intermitente e fadiga. O diagnóstico de síndrome hemofagocítica foi realizado por aspirado de medula óssea, e o tratamento específico foi ineficaz. Trata-se do terceiro caso de síndrome hemofagocítica relacionada a sarcoidose na literatura mundial e o primeiro na literatura latino-americana. Os três casos tiveram desfecho desfavorável. blood workup revealed leukocytosis (neutrophils, 32%). Anemia and thrombocytopenia were found. Electrolytes, creatinine, and urea were normal, and blood and urine cultures were negative (Table 1). The patient was hospitalized for further investigation. He had a medical history of diabetes and dyslipidemia but reported no allergies, blood transfusions, smoking, or alcohol consumption. DescritoresAt admission, the patient was febrile (39°C) and a little anxious. He had no jaundice, rash, or lymphadenopathy. His blood pressure was 130/70 mmHg, his RR was 21 breaths/min, his SaO 2 was 96%, and his HR was 88 bpm. Cardiovascular examination was normal. Pulmonary examination revealed normal breath sounds. Physical examination was otherwise unremarkable. Laboratory test results at admission were similar doses of prednisone (10 mg/day) in order to control the activity of the disease. The patient had been well until March of 2010, when he had episodes of fever. He received emergency room treatment, the amoxicillin-clavulanate combination having been prescribed. The fever did not subside. Ten days later, the patient sought emergency room treatment again (in the same emergency room) and received levofloxacin. The fever persisted, followed by mild dyspnea, a...

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Síndrome hemofagocítica devido a sarcoidose pulmonar

et al. 2012

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“…Monitoring the triglyceride level is a recently defined follow-up parameter in the evaluation of treatment response in secondary HLH as reported by Okamoto et al [17]. Sarper et al [18] reported low triglyceride levels in 2 patients diagnosed with HLH.…”

Section: Discussionmentioning

confidence: 99%

Clinical and laboratory data of primary hemophagocytic lymphohistiocytosis: A retrospective review of the Turkish Histiocyte Study Group

Fışgın¹,

Patıroğlu²,

Ozdemir³

et al. 2010

Turk J Hematol

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Objective: This study analyzes the clinical and laboratory findings of children with primary hemophagocytic lymphohistiocytosis (HLH) followed in various referral centers of Turkey. Materials and Methods: A simple three-page questionnaire prepared by the Turkish Histiocyte Study Group was used for documentation of patient data. Results: Age at diagnosis varied from 0.6 to 78 months (median±SD, 16.5±26.1). Sex distribution was almost equal (F/M=10/12). The frequencies of parental consanguinity and sibling death in the family history were 100% and 81.1%, respectively. The most common clinical findings were hepatomegaly (100%) and fever (95%). The most common laboratory findings were anemia (100%), hyperferritinemia (100%) and thrombocytopenia (90.9%). Triglyceride and total bilirubin levels in the deceased versus surviving group appear to be high (triglyceride: 394±183 mg/dl, 289±7 mg/dl; total bilirubin: 2.7±6.9 mg/dl, 0.5±1.2 mg/ dl, respectively).

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“…24 Triglycerides have been evaluated as a diagnostic and prognostic marker for HLH with good sensitivity. 25 In present study diagnosis of HLH was done based on HLH 2004 criteria. We have not performed genetic and molecular analysis, NK cell activity and soluble IL2 receptor levels because of nonavailability.…”

Section: Diagnostic Criteriamentioning

confidence: 99%

Hemophagocytic lymphohistiocytosis secondary to infections: a tertiary care experience

et al. 2016

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Analysis of Triglyceride Value in the Diagnosis and Treatment Response of Secondary Hemophagocytic Syndrome

Abstract: Background/Aims Secondary hemophagocytic syndrome (hemophagocytic lymphohistiocytosis, HLH)

Cited by 48 publications

References 28 publications

Síndrome hemofagocítica devido a sarcoidose pulmonar

Síndrome hemofagocítica devido a sarcoidose pulmonar

Clinical and laboratory data of primary hemophagocytic lymphohistiocytosis: A retrospective review of the Turkish Histiocyte Study Group

Hemophagocytic lymphohistiocytosis secondary to infections: a tertiary care experience

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