Profile of Hemophagocytic Lymphohistiocytosis; Efficacy of Intravenous Immunoglobulin Therapy

Rajajee, Sarala; Ashok, Indhumathi; Manwani, Nitin; Rajkumar, Jeffrey; Gowrishankar, Kalpana; Subbiah, Ezhilarasi

doi:10.1007/s12098-014-1461-0

Cited by 27 publications

(17 citation statements)

References 18 publications

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“…The study used a preselection of all cases of hyperferritinemia, and control groups did not represent all patients admitted to the MICU. However, previous retrospective data showed that the prevalence of hyperferritinemia in HLH is >90 % (and approaches 100 %) [32,36,37]. Any conclusions regarding relative sensitivity and specificity must be drawn against this cohort selection.…”

Section: Limitationsmentioning

confidence: 98%

Evaluating the optimal serum ferritin level to identify hemophagocytic lymphohistiocytosis in the critical care setting

et al. 2015

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Ferritin is known to be involved in numerous physiological roles, such as iron storage, as well as various pathological conditions and in generalized inflammatory states. Hyperferritinemia is also encountered in the setting of hemophagocytic lymphohistiocytosis (HLH). Current diagnostic criteria exist to define HLH based on several clinical and biochemical markers, including the serum ferritin level. In this study, we retrospectively evaluated the value of ferritin >500 ng/mL in diagnosing HLH in 344 consecutive patients admitted to the medical intensive care unit at our hospital. Nine cases of HLH were identified. Comparison of the HLH with the non-HLH group showed that their maximum median serum ferritin level was 25,652 (range 1977-100,727 ng/mL) versus 1180 (503-85,168 ng/mL) (P < 0.001), platelets were 30 (5-92 × 10(3)/μL) versus 113 (0-507 × 10(3)/μL) (P < 0.001), absolute neutrophil counts were 2.56 (0.02-23.7 × 10(3)/μL) versus 7.7 (0.01-82.7 × 10(3)/μL) (P = 0.002), and triglycerides were 255 (156-394 mg/dL) versus 127 (17-624 mg/dL) (P = 0.002), respectively. Using a receiver operating characteristic curve, the optimal maximum serum ferritin level for the diagnosis of HLH was 3951 ng/mL, exceeding the current diagnostic cutoff set forth in the HLH-2004 guidelines. These data suggest that a higher cutoff value of ferritin level may have improved utility in the diagnosis of secondary HLH in the critical care setting.

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Section: Limitationsmentioning

confidence: 98%

Evaluating the optimal serum ferritin level to identify hemophagocytic lymphohistiocytosis in the critical care setting

et al. 2015

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“…The principal goal of therapy in hyperferritinemic sydromes is to suppress the life-threatening inflammatory process. Hyperferritinemic syndromes (acquired HLH/sepsis/ MODS/MAS) have been successfully treated with intravenous immunoglobulin (IVIg), methylprednisolone, plasma exchange and anakinra [51,[71][72][73]. Indeed, these therapies can be effective modalities, individually or in combination.…”

Section: Managing Cytokine Production And/or Eliminationmentioning

confidence: 99%

“…IVIg is indicated at least theoretically in the view of many mechanism of actions: Fc-receptor blockade, activation of the inhibitory Fcg-receptor IIB, inhibition of complement activation, down regulation of T/B-cell functions, anti-idiotypic suppression, neutralization of superantigens or infectious agents, neutralization of cytokines, enhanced clearance of pathogenic antibodies [77][78][79][80]. The administration of IVIg alone or in combination with other treatment modalities has been described in hyperferritinemic syndromes [71,72]. Variable efficacy is demonstrated, which is not astonishing give the heterogeneity of hyperferritinemic syndromes, the different IVIg preparations on the market, the inherent batch variability, the different dosing schedules, and variability of administration time points used.…”

Section: Ivigmentioning

confidence: 99%

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Management of the critically ill child with the sepsis/hemophagocytic lymphohistiocytosis/macrophage activation syndrome overlap syndrome

Demirkol

Carcillo

2015

J Pediatr Intensive Care

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Hemophagocytic lymphohistiocytosis (HLH) or macrophage activation syndrome (MAS) is a frequently fatal disease, which can result in end-organ damage and death. This condition shares features with sepsis and systemic inflammatory response syndrome. Making a diagnosis of HLH can be challenging since most of the clinical and laboratory features of HLH are quite nonspecific. Timely diagnosis is critical to start therapy before damage by hypercytokinemia becomes irreversible. The treatment for patients with suspected acquired HLH/sepsis/systemic inflammatory response syndrome/multi organ dysfunction syndrome/ MAS overlap syndrome should be guided primarily by the severity of signs and symptoms, age of the patient, and underlying conditions. It is critical that the risks of treatment or non-treatment be weighed according to the clinical presentation of each patient. In this article, the authors discuss the diagnostic similarities between sepsis/HLH/MAS and management of the critically ill child with sepsis/HLH/MAS overlap syndrome.

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“…Y lleva 3 años en remisión, lo que contrasta claramente el pronóstico descrito de SHF de sobrevida media de 2,1 meses en general y 1,4 meses en SHF asociado a neoplasias 19 . Existe alguna evidencia que la administración de IgIV más dexametasona es similar en respuesta al clásico protocolo HLH04 20 .…”

Section: Discussionunclassified

Necrolisis epidérmica tóxica en el curso de un síndrome hemofagocítico secundario a linfoma de Hodgkin. Caso clínico

et al. 2018

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Toxic epidermal necrolysis (TEN) is a lethal entity, characterized by extensive epidermal necrosis and multiorgan failure. Hemophagocytic syndrome (HFS) is also a rare and lethal syndrome characterized by hyperinflammation that leads to the appearance of fever, pancytopenia, organomegaly and hemophagocytosis. The concomitance of these diseases is extremely uncommon. We report a 38 years old female, who during the course of a HFS secondary to Hodgkin Lymphoma (HL), presented a TEN secondary to antibiotics. She was admitted due to a consumptive syndrome, lymphadenopathy, visceromegaly and severe pancytopenia. Laboratory and bone marrow tests confirmed HFS. Due to constant fever, imipenem was indicated. On the third day she started with pain and skin rash. She evolved with positive Nikolsky sign. Cutaneous biopsy was concordant with extensive TEN, which was managed with intravenous immunoglobulin and dexamethasone. A complete response and normalization of the blood count were achieved. Finally, the lymph node biopsy showed HL of mixed cellularity type, which was managed with 8 cycles of ABVD chemotherapy, achieving complete remission.

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Profile of Hemophagocytic Lymphohistiocytosis; Efficacy of Intravenous Immunoglobulin Therapy

Abstract: IVIG and HLH-2004 protocol may be equally effective in the management of HLH. IVIG may be a preferable initial regimen, to avoid the risk of secondary malignancy associated with etoposide.

Cited by 27 publications

References 18 publications

Evaluating the optimal serum ferritin level to identify hemophagocytic lymphohistiocytosis in the critical care setting

Evaluating the optimal serum ferritin level to identify hemophagocytic lymphohistiocytosis in the critical care setting

Management of the critically ill child with the sepsis/hemophagocytic lymphohistiocytosis/macrophage activation syndrome overlap syndrome

Necrolisis epidérmica tóxica en el curso de un síndrome hemofagocítico secundario a linfoma de Hodgkin. Caso clínico

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