Laboratory Examination in Hemophagocytic Lymphohistiocytosis

2021 ◽  
Vol 28 (1) ◽  
pp. 84
Author(s):  
Wulyansari Wulyansari ◽  
Yetti Hernaningsih
Keyword(s):  
Bone Marrow ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Bone Marrow Cells ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Clinical Signs ◽  
Signs And Symptoms ◽  
Laboratory Findings ◽  
Central Nervous System Dysfunction ◽  
Life Threatening ◽  
Clinical Signs And Symptoms

Hemophagocytic Lymphohistiocytosis (HLH) is derived from the word hemophagocytosis, in which macrophagesinfiltrate tissue extensively, and unspecifically phagocyte blood and bone marrow cells. The deviant activation of cytotoxicCD8+ T-cells causing the release of inflammatory cytokines is the core pathogenesis of HLH. Hemophagocyticlymphohistiocytosis is a regulatory disorder of the immune system, with clinical signs and symptoms of extremeinflammation and cytopenia, hepatitis, and severe and life-threatening central nervous system dysfunction. The name of theHLH disorder was recently proposed to be "Hyperinflammatory Lymphohistiocytosis" (also known as HLH). Enforcement ofHLH diagnosis by the Histiocyte Society based on HLH 2004 updated diagnostic criteria consists of five of the following eightdiagnostic criteria: fever, splenomegaly, cytopenia (two or more of three lineages in peripheral blood), hypertriglyceridemiaor hypofibrinogenemia, hyperferritinemia, hemophagocytes in the bone marrow/lien/lymph, the low or non-existentactivity of Natural Killer (NK) cells, increased sCD25. H-score, MH-score, and systemic Juvenile Idiopathic Arthritis(sJIA)/Macrophage Activated Syndrome (MAS) classification criteria are also used to enforce HLH diagnoses.Hemophagocytic lymphohistiocytosis is challenging to recognize and has a high mortality rate, especially in adults, rangingfrom 42 to 88%. Therefore, immediate diagnosis and therapy are essential. The introduction of HLH triggers is criticalbecause treatment is based on the underlying trigger. Cytokine storms due to Coronavirus Disease 19 (COVID-19) infectionhave significant similarities to the clinical and laboratory findings of HLH. Secondary HLH (sHLH) is suspected in severeCOVID-19 patients, so early diagnosis is potentially made based on the H-score.

scholarly journals Successful Modified Therapy in a Patient with Probable Infection-Associated Hemophagocytic Lymphohistiocytosis

2019 ◽  
Vol 2019 ◽  
pp. 1-6
Author(s):  
Carl L. Kay ◽  
Matthew J. Rendo ◽  
Paul Gonzales ◽  
Sead G. Beganovic ◽  
Magdalena Czader
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Clinical Course ◽  
Clinical Signs ◽  
Signs And Symptoms ◽  
Good Patient ◽  
Good Patient Outcome ◽  
Distributive Shock ◽  
Probable Infection ◽  
Clinical Signs And Symptoms ◽  
Baseline Renal Function

Hemophagocytic lymphohistiocytosis (HLH) is a rare, hyperinflammatory syndrome characterized by clinical signs and symptoms of extreme inflammation. In adults, HLH is typically a complication of infections, autoimmune diseases, and malignancies. While the disease is often fatal, classic management of HLH revolves around early diagnosis and initiation of protocolized therapy. We present a case of a previously healthy 56-year-old female who developed distributive shock requiring intubation, vasopressors, and continuous venovenous hemofiltration. In the setting of multiple infectious syndromes, severe cytopenias, and rising direct hyperbilirubinemia, her diagnosis of HLH was confirmed. Therapy was initiated with dexamethasone and two doses of reduced-intensity etoposide based on the patient’s clinical course. Over the next few weeks, she continued to improve on dexamethasone monotherapy and has maintained remission up to the present with complete resolution of her cytopenias and return of baseline renal function. Our case highlights the variability in the management of probable infection-associated HLH (IHLH) with a good patient outcome. We demonstrate the potential to treat IHLH with partial protocols and minimal chemotherapeutics.

scholarly journals Differentiating multisystem inflammatory syndrome in children: a single-centre retrospective cohort study

2021 ◽  
pp. archdischild-2021-322290
Author(s):  
Jordan E Roberts ◽  
Jeffrey I Campbell ◽  
Kimberlee Gauvreau ◽  
Gabriella S Lamb ◽  
Jane Newburger ◽  
...  
Keyword(s):  
Abdominal Pain ◽  
Oral Mucosa ◽  
Multivariable Analysis ◽  
Clinical Signs ◽  
Signs And Symptoms ◽  
Laboratory Findings ◽  
Neutrophil Lymphocyte Ratio ◽  
Lymphocyte Ratio ◽  
Clinical Signs And Symptoms ◽  
Inflammatory Syndrome

ObjectiveFeatures of multisystem inflammatory syndrome in children (MIS-C) overlap with other febrile illnesses, hindering prompt and accurate diagnosis. The objectives of this study were to identify clinical and laboratory findings that distinguished MIS-C from febrile illnesses in which MIS-C was considered but ultimately excluded, and to examine the diseases that most often mimicked MIS-C in a tertiary medical centre.Study designWe identified all children hospitalised with fever who were evaluated for MIS-C at our centre and compared clinical signs and symptoms, SARS-CoV-2 status and laboratory studies between those with and without MIS-C. Multivariable logistic LASSO (least absolute shrinkage and selection operator) regression was used to identify the most discriminative presenting features of MIS-C.ResultsWe identified 50 confirmed MIS-C cases (MIS-C+) and 68 children evaluated for, but ultimately not diagnosed with, MIS-C (MIS-C-). In univariable analysis, conjunctivitis, abdominal pain, fatigue, hypoxaemia, tachypnoea and hypotension at presentation were significantly more common among MIS-C+ patients. MIS-C+ and MIS-C- patients had similar elevations in C-reactive protein (CRP), but were differentiated by thrombocytopenia, lymphopenia, and elevated ferritin, neutrophil/lymphocyte ratio, BNP and troponin. In multivariable analysis, predictors of MIS-C included age, neutrophil/lymphocyte ratio, platelets, conjunctivitis, oral mucosa changes, abdominal pain and hypotension.ConclusionsAmong hospitalised children undergoing evaluation for MIS-C, children with MIS-C were older, more likely to present with conjunctivitis, oral mucosa changes, abdominal pain and hypotension, and had higher neutrophil/lymphocyte ratios and lower platelet counts. These data may be helpful for discrimination of MIS-C from other febrile illnesses, including bacterial lymphadenitis and acute viral infection, with overlapping features.

scholarly journals Comorbidities, clinical signs and symptoms, laboratory findings, imaging features, treatment strategies, and outcomes in adult and pediatric patients with COVID-19: A systematic review and meta-analysis

2020 ◽  
Author(s):  
Catherine Ruth Jutzeler ◽  
Lucie Bourguignon ◽  
Caroline V. Weis ◽  
Bobo Tong ◽  
Cyrus Wong ◽  
...  
Keyword(s):  
Risk Factors ◽  
Systematic Review ◽  
Publication Bias ◽  
Meta Analysis ◽  
Clinical Signs ◽  
Signs And Symptoms ◽  
Imaging Features ◽  
Laboratory Findings ◽  
Clinical Signs And Symptoms ◽  
Meta Analyses

Introduction Since December 2019, a novel coronavirus (SARS-CoV-2) has triggered a world-wide pandemic with an enormous medical, societal, and economic toll. Thus, our aim was to gather all available information regarding comorbidities, clinical signs and symptoms, outcomes, laboratory findings, imaging features, and treatments in patients with coronavirus disease 2019 (COVID-19). Methods EMBASE, PubMed/ Medline, Scopus, and Web of Science were searched for studies published in any language between December 1st, 2019 and March 28th. Original studies were included if the exposure of interest was an infection with SARS-CoV-2 or confirmed COVID-19. The primary outcome was the risk ratio of comorbidities, clinical signs and symptoms, imaging features, treatments, outcomes, and complications associated with COVID-19 morbidity and mortality. We performed random-effects pairwise meta-analyses for proportions and relative risks, I2, Tau2, and Cochrane Q, sensitivity analyses, and assessed publication bias. Results: 148 met the inclusion criteria for the systematic review and meta-analysis with 12149 patients (5739 female) and a median age was 47.0 [35.0-64.6]. 617 patients died from COVID-19 and its complication, while 297 patients were reported as asymptomatic. Older age (SMD: 1.25 [0.78- 1.72]; p < 0.001), being male (RR = 1.32 [1.13-1.54], p = 0.005) and pre-existing comorbidity (RR = 1.69 [1.48-1.94]; p < 0.001) were identified as risk factors of in-hospital mortality. The heterogeneity between studies varied substantially (I2; range: 1.5-98.2%). Publication bias was only found in eight studies (Eggers test: p < 0.05). Conclusions: Our meta-analyses revealed important risk factors that are associated with severity and mortality of COVID-19.

Retrospective Evaluation of Daptomycin, a Lipopeptide Antibiotic, in Bone Marrow Transplant and Hematology/Oncology Patients.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 5531-5531
Author(s):  
Emily R. Stuntebeck ◽  
Daryl D. DePestel ◽  
Curtis D. Collins ◽  
Brian Donovan ◽  
Kenneth C. Lamp
Keyword(s):  
Bone Marrow ◽  
Stem Cell ◽  
Antibiotic Therapy ◽  
Peripheral Blood Stem Cell ◽  
Clinical Signs ◽  
Signs And Symptoms ◽  
Marrow Transplant ◽  
Clinical Signs And Symptoms ◽  
Lipopeptide Antibiotic ◽  
Blood Stem Cell Transplantation

Abstract Background: Daptomycin is a lipopeptide antibiotic with potent activity against many Gram-positive organisms. Efficacy in immunocompromised patients is unknown because these patients have been excluded from daptomycin premarketing studies. Methods: Patients with documented infections treated at the University of Michigan Health System in either the bone marrow transplant or hematology/oncology service were identified in the Cubicin® Outcomes Registry and Experience (COREsm 2004 and 2005). Demographic, disease state, clinical, and microbiologic data were collected. Clinical outcomes were assessed using the following definitions: Cure - clinical signs and symptoms are resolved and/or no additional antibiotic therapy is necessary or infection cleared with a negative culture reported at the end of daptomycin therapy; Improved - partial resolution of clinical signs and symptoms and/or additional antibiotic therapy is necessary at the end of daptomycin therapy; Failure - inadequate response to therapy or resistant, worsening or new/recurrent signs and symptoms, or the need for a change in antibiotic therapy or a positive culture reported at the end of daptomycin therapy; Nonevaluable - unable to determine response at the end of daptomycin therapy. Success was defined as cure or improved. Results: Fourteen patients are included in this analysis. Nine (64%) patients were female; 6 (43%) were ≥ 51 years of age. Two patients had an initial CrCl <30 mL/min, 1 was on hemodialysis. Eight (57%) patients had undergone allogeneic peripheral blood stem cell transplantation primarily for acute myeloid leukemia. The remaining 6 patients had papillary adenocarcinoma, endometrial carcinoma, dermatofibrosarcoma protuberans, acute lymphoblastic lymphoma, non-Hodgkin lymphoma, and Hodgkin lymphoma. The most common infection (n=11, 79%) was bacteremia; 7 (50%) patients had catheter-related bacteremia; 1 each (7%) had discitis, necrotizing fasciitis and urinary tract infection. Nine (64%) patients had vancomycin-resistant Enterococcus faecium (VRE) as a pathogen; 8 were bacteremic (3 with concurrent coagulase-negative staphylococci; CoNS). Methicillin-resistant Staphylococcus aureus (MRSA) was isolated in 3 (21%) patients; 2 were bacteremic (1 with concurrent CoNS). One additional patient had CoNS bacteremia. The patient with necrotizing fasciitis was culture negative. The initial daptomycin dose was 4 mg/kg in 8 (57%) patients and 6 mg/kg in 6 (43%) patients. All patients receiving 6 mg/kg were bacteremic. The dosing frequency was adjusted for renal function in all patients. The median duration of therapy was 14.5 days (range, 2 – 62). Nine (64%) patients received an antibiotic prior to daptomycin and 43% of patients received an antibiotic concomitantly. Seven catheters were removed; 5 from patients with catheter-related bacteremia. The median time to clinical response was 2 days (n=10, range 1 – 13). All patients with an outcome reported (n=11) were successfully treated, 3 (21%) patients were nonevaluable. Of the 11 bacteremic patients, 5 received 4 mg/kg and 6 received 6 mg/kg; 2 were nonevaluable (both 6 mg/kg) and 9 of 11 (82%) were successes. Conclusion: These data demonstrate that daptomycin therapy is associated with clinical success in hem/onc patients including those who have undergone allogeneic peripheral blood stem cell transplantation where bacteremia and VRE are prevalent.

Bone marrow transplantation for childhood Ki-1 lymphoma.

1990 ◽  
Vol 8 (4) ◽  
pp. 657-660 ◽  
Author(s):  
V Chakravarti ◽  
N R Kamani ◽  
E Bayever ◽  
B Lange ◽  
P Herzog ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Transplantation ◽  
Marrow Transplantation ◽  
Allogeneic Bone Marrow Transplantation ◽  
Clinical Signs ◽  
Signs And Symptoms ◽  
High Dose Chemotherapy ◽  
High Dose ◽  
Allogeneic Bone ◽  
Clinical Signs And Symptoms

Two children with Ki-1 antigen-positive, non-Hodgkin's lymphoma received high-dose chemotherapy, fractionated total body irradiation (TBI), and allogeneic bone marrow transplantation. Both patients had relapsed multiple times on conventional chemotherapy and radiation therapy. Following transplantation, there was successful engraftment with disappearance of clinical signs and symptoms of their disease. As of June 1, 1989 they are in continuous unmaintained complete remission, 56 and 40 months, respectively, after bone marrow transplantation.

scholarly journals A Brief Review on Covid-19 Effects in Children and Treatment Methods

2021 ◽  
Vol 70 (1) ◽  
Author(s):  
Vaishnavi Tammishetty ◽  
Sravika Nagamalla ◽  
Pravalika Sakilam ◽  
Hyma Ponnaganti
Keyword(s):  
Clinical Signs ◽  
Clinical Manifestations ◽  
Medical Practitioner ◽  
Signs And Symptoms ◽  
Limited Data ◽  
Laboratory Findings ◽  
Gi Symptoms ◽  
Review Study ◽  
Clinical Signs And Symptoms ◽  
Inflammatory System

As there is an outbreak of novel corona virus in 2019 it has spread globally that resulted in severe acute respiratory syndrome coronavirus 2 (SARS-COV-2) pandemic and mostly there is limited data provided on children. The main aim of this review is to provide a knowledge on introduction, epidemiology, pathogenesis, transmission, clinical manifestations, laboratory findings, treatment of COVID-19 in children. And it also includes latest statistical data of children prone to COVID-19. Besides respiratory and GI symptoms atypical features such as chilblains and multi-inflammatory system are also reported. pathophysiology gives information regarding the life cycle of virus in hostcell and epidemiology explains the different types of viruses affecting the respiratory system. The clinical signs and symptoms are almost similar to the adults but they are in mild, and most of the children affected with Covid-19 are asymptomatic. This review study makes a medical practitioner to have a quick, practical approach to the disease to use in different scopes, especially in pediatric medicine.

scholarly journals Autosomal recessive infantile osteopetrosis: case report with radiological review

2017 ◽  
Vol 5 (6) ◽  
pp. 2797
Author(s):  
Tushar Kalekar ◽  
Vipul Sehrawat
Keyword(s):  
Bone Marrow ◽  
Ethnic Groups ◽  
Autosomal Recessive ◽  
Bone Marrow Failure ◽  
Clinical Signs ◽  
Signs And Symptoms ◽  
Female Child ◽  
Extramedullary Hematopoiesis ◽  
Marrow Cavity ◽  
Clinical Signs And Symptoms

Autosomal recessive type of osteopetrosis or infantile malignant osteopetrosis is a rare congenital disorder of bone resorption characterised by generalised skeletal densification. Incidence is estimated around 1/2,00,000 live births. Osteopetrosis has been reported in most ethnic groups, although, as the disease is very rare, it is more frequently seen in ethnic groups where consanguinity is common. Bone marrow failure, fractures and visual impairment are the classical features of the disease, which begins in early infancy or in foetal life. It results from the failure of osteoclasts to resorb immature bone. This leads to abnormal bone marrow cavity formation and to the clinical signs and symptoms of bone marrow failure. It is accompanied by hepatosplenomegaly due to compensatory extramedullary hematopoiesis. Because of rarity of this type of osteopetrosis, we would like to report this case of a female child who presented with cough, fever and anemia at the age of 3 years.

Cardiac Tamponade Physiology Secondary to Tense Ascites

Cardiology ◽  
2016 ◽  
Vol 134 (4) ◽  
pp. 423-425 ◽  
Author(s):  
Saad Ahmad ◽  
Hina K. Jamali ◽  
Fahad Waqar ◽  
David M. Harris
Keyword(s):  
Liver Disease ◽  
Cardiac Tamponade ◽  
Clinical Signs ◽  
Signs And Symptoms ◽  
Abdominal Pressure ◽  
Inferior Wall ◽  
Extrinsic Compression ◽  
Life Threatening ◽  
Clinical Signs And Symptoms ◽  
High Index Of Suspicion

Cardiac tamponade is a common and often life-threatening process, which is typically associated with a pericardial effusion or, in rare cases, with a large pleural effusion. Theoretically, as reported in only a single prior case, it can be caused by extrinsic compression from tense ascites. We present a case in which dynamic inferior wall collapse was secondary to increased abdominal pressure from tense ascites. This phenomenon may be more common than previously diagnosed, especially in patients with liver disease. These patients often develop frequent ascites and present with clinical signs and symptoms similar to cardiac tamponade (tachycardia, hypotension and dyspnea). Presently, no formal practice guidelines exist regarding cardiac imaging for these patients. A high index of suspicion is required for timely diagnosis and management.

Serotonin toxicity

2011 ◽  
Vol 28 (2) ◽  
pp. i-iv ◽  
Author(s):  
Sobia Nasim ◽  
Faraz Jabbar ◽  
Asfar Afridi ◽  
Brendan D Kelly
Keyword(s):  
Differential Diagnosis ◽  
Clinical Signs ◽  
Signs And Symptoms ◽  
Causal Agent ◽  
Vital Functions ◽  
Threatening Condition ◽  
Life Threatening ◽  
Clinical Signs And Symptoms ◽  
High Index Of Suspicion ◽  
Serotonin Toxicity

Serotonin toxicity is a potentially life-threatening condition associated with a range of psychotropic medications, co-administration of specific combinations of agents and overdose of certain drugs. It is associated with a wide diversity of clinical signs and symptoms, including cognitive, autonomic and somatic effects, as well as serious complications, including possible death. Diagnosis is often challenging and requires a high index of suspicion. Differential diagnosis includes syndromes such as neuroleptic malignant syndrome. Management depends on the causal agent and urgency of clinical presentation. Treatment may involve discontinuing the causal agent and providing supportive measures, or emergency intervention to preserve vital functions (airway, breathing, circulation), amongst other measures. Further research is needed to clarify the incidence of serotonin toxicity, issues related to differential diagnosis, optimal management of the condition, and treatment of mood problems following serotonin toxicity.

Vemurafenib-induced pancreatitis in a patient with recurrent hairy cell leukaemia

2020 ◽  
Vol 13 (9) ◽  
pp. e236073
Author(s):  
Su Yun Chung ◽  
Janice Gloria Shen ◽  
Cristina M Ghiuzeli
Keyword(s):  
Clinical Signs ◽  
Signs And Symptoms ◽  
Interesting Case ◽  
Hairy Cell Leukaemia ◽  
Cell Leukaemia ◽  
Hairy Cell ◽  
Braf Inhibitors ◽  
Severe Pancreatitis ◽  
Life Threatening ◽  
Clinical Signs And Symptoms

Recent studies have shown that BRAF inhibitors, such as vemurafenib, are effective in inducing long periods of remission in relapsed hairy cell leukaemia. Acute pancreatitis is one of the rare complications that is reported with vemurafenib use. As severe pancreatitis can be life threatening, physicians should be vigilant of this side effect and promptly treat patients that develop clinical signs and symptoms while receiving vemurafenib. We present an interesting case of vemurafenib-induced pancreatitis that not only resolved but also did not recur after reintroduction of the drug at a reduced dose.

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