scholarly journals IMPORTANCE OF HYPERBILURUBINEMIA IN DIFFERENTIATION OF PRIMARY AND SECONDARY HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN PEDIATRIC CASES

2014 ◽  
Vol 6 (1) ◽  
pp. e2014067 ◽  
Author(s):  
Seval Ozen ◽  
Alper Dai ◽  
Enes Coskun ◽  
Serdar Oztuzcu ◽  
Sercan Ergun ◽  
...  
Keyword(s):  
Inflammatory Disease ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Clinical Findings ◽  
Initial Diagnosis ◽  
Direct Bilirubin ◽  
Pediatric Hematology ◽  
Life Threatening ◽  
Secondary Hemophagocytic Lymphohistiocytosis

Background and objective: Hemophagocytic lymphohistiocytosis (HLH) is a life threatening hyper inflammatory disease. It is difficult to differentiate between primary and secondary HLH based on clinical findings at the onset of disease. We aimed to find parameters that can help to differentiate primary and secondary HLH at initial diagnosis especially for physicians working in developing countries.Patient and Method: We retrospectively analyzed data of 38 HLH patients who were admitted to the Pediatric Hematology Department of Gaziantep University between January 2009 and December 2013.Results: Of 38 patients, 20 were defined as primary and 18 were secondary HLH. The average age of primary and secondary HLH patients was 31±9 and 81±14 months, respectively (p=0.03). We found consanguinity rates significantly higher in primary HLH patients compared to secondary HLH patients (p=0.03). We found that total and direct bilirubin levels significantly increased in primary HLH patients compared to secondary HLH patients (p=0.006, p=0.044). Also, CRP levels were found markedly increased in secondary HLH patients compared to primary ones (p=0.017).Conclusion: We showed that cholestasis and hyperbilurubinemia findings of HLH patients at the initial diagnosis should be considered in favor of primary HLH and increased level of CRP should be considered in favor of secondary HLH.

scholarly journals Hemophagocytic lymphohistiocytosis: a rare and life-threatening diagnosis

2021 ◽  
Vol 9 (12) ◽  
pp. 3705
Author(s):  
Anu Yarky ◽  
Vipan Kumar ◽  
Nidhi Chauhan ◽  
Neha Verma
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Clinical Presentation ◽  
Laboratory Findings ◽  
Delay In Diagnosis ◽  
Life Threatening ◽  
Sporadic Cases ◽  
Prompt Diagnosis ◽  
Excessive Activation ◽  
Variable Clinical Presentation ◽  
Secondary Hemophagocytic Lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome of excessive activation of immune system. It frequently affects infants from birth to 18 months of age, but is also observed in children and adults of all ages. HLH can occur as a familial or sporadic disorder, and it is triggered by a variety of events, Infection being the most common trigger both in familial and in sporadic cases. Prompt treatment is very critical in cases of HLH, but the greatest barrier is often delay in diagnosis due to the rarity of this syndrome, variable clinical presentation, and lack of specificity of the clinical and laboratory findings. The key clinical features of HLH are high persistent fever, hepatosplenomegaly, blood cytopenia, elevated aminotransferase and ferritin levels, and coagulopathy. A diagnosis of HLH is mostly under-recognized, and is associated with high mortality, especially in adults; thus, prompt diagnosis and treatment are essential. We here present a rare case of HLH in an adult which was non-familial and infection being the trigger causing secondary hemophagocytic lymphohistiocytosis.

ADULT SECONDARY HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS – A CASE SERIES

2021 ◽  
pp. 64-66
Author(s):  
Athira P A ◽  
Indumathi K DCP ◽  
Theranirajan Theranirajan ◽  
Priyadharshini Swaminathan
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Macrophage Activation ◽  
Case Series ◽  
Treatment Phase ◽  
Marrow Transplant ◽  
Allogenic Bone ◽  
Familial Form ◽  
Life Threatening ◽  
Antigen Removal ◽  
Secondary Hemophagocytic Lymphohistiocytosis

Hemophagocytic lymphohistiocytosis is a hyper-inammatory condition that is either Familial (Primary) or Secondary to autoimmune diseases , infection, malignancy or other triggers.It is a cytokine storm syndrome where there inefcient antigen removal that leads to sustained cytokine release.It is a rare phenomenon occuring in adults that has got a specic trigger which is less documented and have a good response to steroids where as Familial form is a childhood disease due to genetic defects, both of which are life threatening and may need Allogenic bone marrow transplant. Macrophage activation syndrome is also a subtype of this entity that occurs in the treatment phase of SLE and Still's disease.We describe here 8 cases of secondary HLH, their primary triggers and treatment response.

Hemophagocytic lymphohistiocytosis (HLH) and related disorders

Hematology ◽  
2009 ◽  
Vol 2009 (1) ◽  
pp. 127-131 ◽  
Author(s):  
Alexandra H. Filipovich
Keyword(s):  
T Cells ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Hematopoietic Cell Transplantation ◽  
Viral Infections ◽  
Clinical Findings ◽  
Antiinflammatory Therapy ◽  
Genetic Causes ◽  
Life Threatening ◽  
Genetically Determined ◽  
Antigen Presenting

Abstract Hemophagocytic lymphohistiocytosis (HLH), which has many genetic causes, is characterized by multi-system inflammation. HLH is a reactive process resulting from prolonged and excessive activation of antigen presenting cells (macrophages, histiocytes) and CD8+ T cells. Hemophagocytosis, which is mediated through the CD163 heme-scavenging receptor, is a hallmark of activated macrophages/histiocytes and is the characteristic finding for which the disorder was named. The majority of genetic causes identified to date affect the cytotoxic function of NK and T cells, crippling immunologic mechanisms that mediate natural immune contraction. The predominant clinical findings of HLH are fevers (often hectic and persistent), cytopenias, hepatitis and splenomegaly. Due to the life-threatening implications of the diagnosis of genetically determined HLH, antiinflammatory therapy, often consisting of steroids, etoposide or antithymocyte globulin (ATG), should be instituted promptly, followed by curative hematopoietic cell transplantation. Secondary HLH, associated with autoimmune disorders or viral infections in teens and adults, also carries a significant mortality rate and should be managed in consultation with specialists familiar with the diagnosis and treatment of such disorders.

scholarly journals Risk factors for secondary hemophagocytic lymphohistiocytosis in severe coronavirus disease 2019 adult patients

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Mei Meng ◽  
Limin Chen ◽  
Sheng Zhang ◽  
Xuan Dong ◽  
Wenzhe Li ◽  
...  
Keyword(s):  
Risk Factors ◽  
Organ Failure ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Viral Infections ◽  
Adult Patients ◽  
Reactive Protein ◽  
Failure Assessment ◽  
Life Threatening ◽  
Independent Risk Factors ◽  
Secondary Hemophagocytic Lymphohistiocytosis

Abstract Background Secondary hemophagocytic lymphohistiocytosis (sHLH) is a life-threatening hyperinflammatory event and a fatal complication of viral infections. Whether sHLH may also be observed in patients with a cytokine storm induced by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is still uncertain. We aimed to determine the incidence of sHLH in severe COVID-19 patients and evaluate the underlying risk factors. Method Four hundred fifteen severe COVID-19 adult patients were retrospectively assessed for hemophagocytosis score (HScore). A subset of 7 patients were unable to be conclusively scored due to insufficient patient data. Results In 408 patients, 41 (10.04%) had an HScore ≥169 and were characterized as “suspected sHLH positive”. Compared with patients below a HScore threshold of 98, the suspected sHLH positive group had higher D-dimer, total bilirubin, alanine aminotransferase, aspartate aminotransferase, blood urea nitrogen, serum creatinine, triglycerides, ferritin, interleukin-6, C-reactive protein, procalcitonin, lactate dehydrogenase, creatine kinase isoenzyme, troponin, Sequential Organ Failure Assessment (SOFA) score, while leukocyte, hemoglobin, platelets, lymphocyte, fibrinogen, pre-albumin, albumin levels were significantly lower (all P < 0.05). Multivariable logistic regression revealed that high ferritin (>1922.58 ng/mL), low platelets (<101 × 109/L) and high triglycerides (>2.28 mmol/L) were independent risk factors for suspected sHLH in COVID-19 patients. Importantly, COVID-19 patients that were suspected sHLH positive had significantly more multi-organ failure. Additionally, a high HScore (>98) was an independent predictor for mortality in COVID-19. Conclusions HScore should be measured as a prognostic biomarker in COVID-19 patients. In particular, it is important that HScore is assessed in patients with high ferritin, triglycerides and low platelets to improve the detection of suspected sHLH.

scholarly journals Prevalence and Outcome of Secondary Hemophagocytic Lymphohistiocytosis Among SIRS Patients: Results from a Prospective Cohort Study

2019 ◽  
Vol 8 (4) ◽  
pp. 541 ◽  
Author(s):  
Gualdoni ◽  
Hofmann ◽  
Wohlfarth ◽  
Winkler ◽  
Winkler ◽  
...  
Keyword(s):  
Cohort Study ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Underlying Disease ◽  
Observational Cohort Study ◽  
Point Increase ◽  
Threatening Condition ◽  
Life Threatening ◽  
Observational Cohort ◽  
Sirs Criteria ◽  
Secondary Hemophagocytic Lymphohistiocytosis

Secondary hemophagocytic lymphohistiocytosis (sHLH) is a life-threatening condition clinically presenting as SIRS (Systemic Inflammatory Response Syndrome). However, there is no comprehensive data concerning diagnostic algorithms, prevalence, outcome and biomarker performance in SIRS patients. We conducted a prospective observational cohort study on 451 consecutive patients fulfilling ≥2 SIRS criteria. The Hscore and the HLH-2004 criteria were used to determine the presence of sHLH, and the correlation of the screening-biomarkers ferritin, sCD25, and sCD163 with both scores was assessed. Out of 451 standard-care SIRS patients, five patients had high Hscores (≥169), suggesting incipient or HLH-like disease, and these patients were in urgent need for intensified therapy. However, none of these patients fulfilled five HLH-2004 criteria required for formal diagnosis. From the studied biomarkers, ferritin correlated strongest to both the HLH-2004 criteria and the Hscore (rs = 0.72, 0.41, respectively), and was the best predictor of 30-day survival (HR:1.012 per 100 μg/L, 95% CI: 1.004–1.021), when adjusted for patient’s age, sex, bacteremia and malignant underlying-disease. Also, the HLH-2004 (HR per point increase: 1.435, 95% CI: 1.1012–2.086) and the Hscore (HR per point increase:1.011, 95% CI: 1.002–1.020) were independent predictors of 30-day-survival. The Hscore detected patients in hyperinflammatory states requiring urgent therapy escalation. Degrees of hyperinflammation, as assessed by ferritin and both HLH scores, are associated with worse outcomes.

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