Reactive Hemophagocytic Syndrome Associated With Disseminated Strongyloidiasis

Finkielman, Javier D.; Grinberg, Alejandro R.; Paz, Leonardo; Plana, Jose L.; Benchetrit, Guillermo; Nicastro, Mario A.; Aj, Roncoroni

doi:10.1097/00000441-199607000-00008

Cited by 16 publications

(9 citation statements)

References 6 publications

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“…Secondary HLH consists of a variety of conditions associated with infection [6], malignancy [5], autoimmune disorder [7,8], and drugs [9]. Therefore, its clinical manifestation, course, and treatment response are not only affected by HLH itself, but are also related to possible underlying conditions such as lymphoma [10].…”

Section: Introductionmentioning

confidence: 99%

Clinical features of adult patients with secondary hemophagocytic lymphohistiocytosis from causes other than lymphoma: an analysis of treatment outcome and prognostic factors

et al. 2011

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Although hemophagocytic syndrome (HS) featuring secondary hemophagocytic lymphohistiocytosis (HLH) has a grave prognosis, little is known about the natural course of the disease. Patients who showed the clinical features of HLH as well as tissue-proven hemophagocytosis when seen at Asan Medical Center between 1999 and 2010 were included in this analysis. Patients with proven lymphoma were excluded. The median age of our 23 study patients was 49 years. Epstein-Barr virus was suspected to have caused HS in 16 (70%) patients and hepatitis A virus in one patient. Twenty-two patients were treated, 13 according to the HLH protocol and nine using immunosuppressive agents such as corticosteroid and/or cyclosporine. Five patients undertook allogeneic hematopoietic cell transplantation (HCT) during their treatment-dependent relapse (n = 4) or responsive status (n = 1). After the median follow-up of 180 days, 17 (74%) died and six (26%) were alive. The median time from initial presentation until death was 41 days among those patients who died. The serum fibrinogen level ≥166 mg/dL determined at the initial visit was significantly associated with the survival time according to univariate analysis. The low histiocyte proportion in bone marrow and early initiation of treatment tended to correlate with a favorable outcome. On multivariate analysis, serum fibrinogen ≥166 mg/dL (hazard ratio, 0.175, P = 0.018) was an independent clinical factor for determining the patient survival time. Despite appropriate patient management, the outcome of HS featuring HLH was grave. The serum fibrinogen level at the initial presentation was significant, and selected patients obtained some benefit from allogeneic HCT.

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Section: Introductionmentioning

confidence: 99%

Clinical features of adult patients with secondary hemophagocytic lymphohistiocytosis from causes other than lymphoma: an analysis of treatment outcome and prognostic factors

et al. 2011

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“…Secondary HLH is an aetiologically heterogeneous entity. It can be associated various infections [9][10][11], haematological malignancies [12], autoimmune disorders [13,14] and drugs [15]; and is therefore more prevalent than primary HLH. Given the diverse biological, clinical signs and laboratory abnormalities; secondary HLH is often misdiagnosed or altogether unrecognized, resulting in high mortality rates.…”

Section: Introductionmentioning

confidence: 99%

Secondary hemophagocytic syndrome in adults: a case series of 18 patients in a single institution and a review of literature

Shabbir

Lucas

Liu

et al. 2010

Hematological Oncology

102

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Hemophagocytic lymphohistiocytosis (HLH) is rare in adults and is usually fatal without treatment. We present a consecutive series of 18 adults with HLH diagnosed at our institution between 2004 and 2009. All diagnoses were confirmed by pathology. The median age at diagnosis was 56 years (range: 18-73 years), with a male: female ratio of 2:1. Patients uniformly presented with fever. Fifty-five per cent of the patients presented with evidence of hepatomegaly or splenomegaly. All of the patients had at least a bi- or trilineage cytopenia. Elevated liver enzymes, hyperferritinemia, hypertriglyceridemia and hyperfibrinogenemia were seen in 50, 100, 40 and 50% of patients, respectively. The presumed causes were as follows; haematological malignancies (n = 4), post-autologous stem cell transplant (n = 2), infection (n = 2), rheumatologic illness (n = 2), sickle cell disease (n = 1), post-orthotopic liver transplant (n = 1) and idiopathic (n = 3). The median time from suspicion to diagnosis was 5 days (1-27 days). Corticosteroids and/or cyclosporine were the most frequently used treatment regimen. Other agents used were etoposide, IVIG, cyclophosphamide and chemotherapy. The mortality rate was 72%, with multi-system organ failure being the most common cause of death. Median survival time from diagnosis was 35 days. Six patients are alive to date. In a univariate analysis, the presence of fever was the only factor that was statistically significant for predicting a poor prognosis (early mortality) (p = 0.05). In conclusion, a high index of suspicion is the critical factor for early diagnosis. Early treatment with immunosuppressant is warranted, and a thorough diagnostic evaluation to identify the underlying cause should be undertaken.

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“…[8][9][10][11][12][13][14][15][16][17] The duration of SLE before the onset of strongyloidiasis was variable (5 weeks to 12 years), but all of the patients reported therapeutic contents were using corticosteroids. Symptoms that resembled those of Strongyloidiasis causes anemia, eosinophilia, hypocholesterolemia (due to abnormal fat absorption), and hypoalbuminemia (due to protein-losing enteropathy).…”

Section: Discussionmentioning

confidence: 99%

Recurrent paralytic ileus associated with strongyloidiasis in a patient with systemic lupus erythematosus

et al. 2006

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We present an interesting case of recurrent paralytic ileus due to strongyloidiasis in a woman who was being treated with corticosteroids and immunosuppressants for systemic lupus erythematosus (SLE). She was also a carrier of human T-cell leukemia virus type I. She had a history of strongyloidiasis 8 years earlier. Recurrent episodes of paralytic ileus due to strongyloidiasis occurred during treatment of her SLE with corticosteroids. Ivermectin was given and improved the symptoms. This case shows that symptomatic strongyloidiasis can be induced in immunocompromised hosts by immunosuppressive therapy. It is important to rule out strongyloidiasis prior to starting immunosuppressive therapy in patients from endemic areas.

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Reactive Hemophagocytic Syndrome Associated With Disseminated Strongyloidiasis

Cited by 16 publications

References 6 publications

Clinical features of adult patients with secondary hemophagocytic lymphohistiocytosis from causes other than lymphoma: an analysis of treatment outcome and prognostic factors

Clinical features of adult patients with secondary hemophagocytic lymphohistiocytosis from causes other than lymphoma: an analysis of treatment outcome and prognostic factors

Secondary hemophagocytic syndrome in adults: a case series of 18 patients in a single institution and a review of literature

Recurrent paralytic ileus associated with strongyloidiasis in a patient with systemic lupus erythematosus

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