scholarly journals Hemophagocytic lymphohistiocytosis and myelodysplastic syndrome: a case report and review of the literature

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Y. Sun ◽  
C. Blieden ◽  
B. Y. Merritt ◽  
R. Sosa ◽  
Gustavo Rivero
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Clinical Laboratory ◽  
Hematologic Malignancies ◽  
Rapid Onset ◽  
Rapid Identification ◽  
African American Patient ◽  
Trisomy 8 ◽  
American Patient ◽  
Life Threatening ◽  
Therapy Design

Abstract Background Hemophagocytic lymphohistiocytosis (HLH) is characterized by hyperinflammation and life-threatening cytopenias. Survival is poor, and management is pivotal on rapid identification of the disease. HLH is associated with hematologic malignancies, however correlation with myelodysplastic syndromes (MDS) is exceedingly unusual. Although minimizing overwhelming hyperinflammation by treating hemophagocytosis are central for HLH outcome, there is urgent necessity to identify potential initiating mechanisms that could assist in therapy design. Case description Here, we describe an elderly African American patient who developed rapid onset of cytopenias and coagulopathy associated with hepatic and bone marrow hemophagocytosis. We analyze four additional similar cases to isolate clinical, laboratory and cytogenetic findings expected in patients exhibiting concurrent HLH and MDS. HLH linked with MDS retains common HLH features associated with systemic hyperinflammation such as fever, hypotension, hepatosplenomegaly, hyperferritinemia, coagulopathy and rapidly evolving cytopenias. Typical MDS chromosomic abnormality such as trisomy 8 was frequently observed in our studied cases. Conclusion Our case describes difficulties while managing HLH in MDS patients. Diagnosis should be based on identifying HLH appropriate criteria and if possible karyotypic abnormalities normally observed in MDS.

A Test for Bacterial Alkaline Phosphatase: Use in Rapid Identification of Serratia Organisms

1973 ◽  
Vol 19 (11) ◽  
pp. 1248-1249 ◽  
Author(s):  
Paul L Wolf ◽  
Elisabeth Von der Muehll ◽  
Karen Praisler
Keyword(s):  
Alkaline Phosphatase ◽  
Clinical Laboratory ◽  
Rapid Identification ◽  
Hospitalized Patients ◽  
Coagulase Negative Staphylococcus ◽  
Blue Color ◽  
Bacterial Alkaline Phosphatase ◽  
Life Threatening ◽  
Life Threatening Complication ◽  
Coagulase Positive Staphylococcus

Abstract This investigation concerns identification of alkaline phosphatase production by bacterial organisms, as detected by a blue color resulting from conversion of indolyl phosphate to indigo. Coagulase-positive Staphylococcus produced alkaline phosphatase; coagulase-negative Staphylococcus did not. Serratia did not produce alkaline phosphatase; those Enterobacteriaceae we tested did. Thus, this test rapidly differentiates these organisms, diminishing the time for identification of Serratia in the clinical laboratory by 48 h. Identification of Serratia should not be ignored, because it is a life-threatening complication for certain hospitalized patients.

scholarly journals Parameters for therapeutic laparotomy in blunt trauma abdomen

2018 ◽  
Vol 6 (1) ◽  
pp. 159
Author(s):  
Younis Ahmad ◽  
Arshid Iqbal Qadri ◽  
Iqtibas Ahmad Ganie ◽  
Muqtasid Rashid ◽  
Gowhar Aziz Bhat
Keyword(s):  
Blunt Trauma ◽  
Scoring System ◽  
Clinical Laboratory ◽  
Recursive Partitioning ◽  
Operative Management ◽  
Rapid Identification ◽  
Large Sample Size ◽  
Radiological Parameters ◽  
Life Threatening ◽  
Non Operative Management

Background: The rapid identification of potentially life threatening intra-abdominal injury is critical for patients who sustain blunt abdominal trauma. There has been a shift from operative to non-operative management (NOM) in hemodynamically stable blunt trauma abdomen patients. The aim of present study was to determine the certain clinical and radiological parameters for therapeutic laparotomy (TL) in blunt trauma abdomen in adult patients.Methods: A prospective observational study was conducted on victims of blunt trauma who presented to our level II Emergency Department from May 2012 to June 2014. Their clinical, laboratory and radiological parameters were collected, evaluated and analyzed. A previously developed ultrasound scoring system was applied to FAST findings. Patients were followed to determine if they underwent TL or NOM.Results: A total of 7750 polytrauma patients with suspected blunt trauma abdomen underwent FAST. 338 (4.36%) patients had a positive FAST, out of which 144 were included, 93 (64.58%) patients were selected for NOM, and 51 (35.4%) patients underwent TL. NOM was successful in 76 (81.73%) patients, whereas 17 (18.27%) failed NOM and were operated. Using recursive partitioning analysis, the most important predictor for a TL, was whether the patient has an ED SBP of ≤90mmHg while other parameters include, ED pulse rate >110/min; total fast score >3; large amount of hemoperitoneum; presence of abdominal guarding, pallor and polytrauma.Conclusions: There are certain immediately available clinical, and radiological parameters, which if validated by a prospective, large sample size study could help in deriving a decision rule or even a scoring system that would determine the need for therapeutic laparotomy in blunt trauma abdomen patients.

Serum soluble CD25 and ferritin in distinguishing patients with uncomplicated hematologic malignancies from patients with hematologic malignancies complicated by hemophagocytic lymphohistiocytosis.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e20072-e20072
Author(s):  
Adi Zoref Lorenz ◽  
Liron Hofstetter ◽  
Jun Murakami ◽  
Oren Pasvolsky ◽  
Elad Guber ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Nk Cell ◽  
Serum Levels ◽  
Hematologic Malignancies ◽  
Screening Criteria ◽  
Underlying Malignancy ◽  
Life Threatening ◽  
Sensitivity Specificity ◽  
Inflammatory Syndrome

e20072 Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyper-inflammatory syndrome with distinct clinical and laboratory features. In adults, HLH is often associated with underlying malignancy, most commonly hematologic malignancies (HM). HLH occurs in 1% of adults with HM and overall survival can be low as 10-20%. Abnormal serum levels of the inflammatory markers sCD25 and ferritin are diagnostic criteria for familial HLH. However, because these reactants are often elevated in malignancy, appropriate levels for diagnosis in HM-HLH are unknown. In this study, we establish optimal sCD25 and ferritin levels for the diagnosis of HM-HLH in adults. Methods: Patients from three centers in Israel and Japan with HM-HLH and HM in whom sCD25 testing was performed were studied. The diagnosis of HLH was according to the HLH 2004 diagnostic criteria. Initial (at HLH presentation) and the maximum ferritin levels were analyzed. Sensitivity, specificity, and optimal cutoffs were calculated by receiver operating characteristic (ROC). Results: 62 patients with HM's without HLH and 40 patients with HM-HLH were included. The distribution of ages and HM subtypes was similar between groups (mostly B cell, T/NK cell, and Hodgkin's lymphoma). The median sCD25 concentration in HM was 1776 U/ml versus 8077 U/ml in HM-HLH. The median initial/ maximum ferritin levels were 190/202 ng/ml for the HM group and 2267/4515 ng/ml for the HM-HLH group. Both sCD25 and ferritin were very sensitive but nonspecific. sCD25 > 2400 U/ml had a sensitivity/specificity of 95%/65%, while initial ferritin > 500 ng/ml had a sensitivity/specificity of 95%/75%. ROC analysis demonstrated optimal confirmatory cutoff values (maximizing specificity) of > 10,056 ng/ml for sCD25 (sensitivity/specificity 47%/95%). While initial ferritin demonstrated a cutoff of > 5231 ng/ml (sensitivity/specificity 22.5%/95%, AUC = 0.88) the maximum ferritin performed better with the cutoff of > 5748 ng/ml (sensitivity/specificity 45%/95%, AUC = 0.92). Conclusions: Our data suggest that the current HLH 2004 criteria of ferritin > 500 ng/ml and sCD25 > 2400 U/ml are effective screening criteria for the complication of HLH in HM patients. sCD25 > 10,000 U/ml and initial ferritin > 5,200 ng/ml are highly specific. Patients with suspected HM- HLH should have serial ferritin testing which increases specificity of this test. Future prospective studies are needed to confirm these findings.

scholarly journals Imatinib-Induced Acquired Dermal Melanocytosis

2021 ◽  
Vol 5 (1) ◽  
pp. 75-77
Author(s):  
Anna Eversman ◽  
Sakeena Fatima ◽  
Kord Honda ◽  
Mara Beveridge
Keyword(s):  
Kinase Inhibitor ◽  
Lymphoblastic Leukemia ◽  
Hematologic Malignancies ◽  
Imatinib Treatment ◽  
African American Patient ◽  
Early Referral ◽  
Case Presentation ◽  
Stromal Tumors ◽  
American Patient ◽  
Increased Risk

Background: Imatinib, a tyrosine kinase inhibitor targeting bcr-abl, c-Kit, and PDGF receptors-alpha and beta, is commonly used to treat GI stromal tumors and hematologic malignancies. Hyperpigmentation is a known side-effect of imatinib, with intradermal hemosiderosis being the most common histologic finding. Case Presentation: We report a rare case of concurrent hypopigmentation and hyperpigmentation secondary to dermal melanocytosis following imatinib treatment in an African American patient with acute lymphoblastic leukemia. Conclusion: Early referral to dermatology in patients with similar findings is appropriate given the increased risk of melanoma conferred by heightened melanocyte proliferation.

scholarly journals Anaphylactic Death: A New Forensic Workflow for Diagnosis

Healthcare ◽  
2021 ◽  
Vol 9 (2) ◽  
pp. 117
Author(s):  
Massimiliano Esposito ◽  
Angelo Montana ◽  
Aldo Liberto ◽  
Veronica Filetti ◽  
Nunzio Di Nunno ◽  
...  
Keyword(s):  
Clinical History ◽  
Rapid Onset ◽  
Mean Value ◽  
Laryngeal Edema ◽  
Tryptase Level ◽  
Antibody Staining ◽  
Life Threatening ◽  
History Of ◽  
Immunohistochemical Techniques

Anaphylaxis is a life-threatening or fatal clinical emergency characterized by rapid onset, and death may be sudden. The margin of certainty about the diagnosis of anaphylactic death is not well established. The application of immunohistochemical techniques combined with the evaluation of blood tryptase concentrations opened up a new field of investigation into anaphylactic death. The present study investigated eleven autopsy cases of anaphylactic death, carried out between 2005 and 2017, by the Departments of Forensic Pathology of the Universities of Foggia and Catania (Italy). An analysis of the medical records was carried out in all autopsies. Seven autopsies were carried out on males and four on females. Of the eleven cases, one showed a history of asthma, one of food ingestion, two of oral administration of medications, six did not refer any allergy history, and one subject was unknown. All cases (100%) showed pulmonary congestion and edema; 7/11 (64%) of the cases had pharyngeal/laryngeal edema and mucus plugging in the airway; only one case (9%) had a skin reaction that was found during external examination. Serum tryptase concentration was measured in ten cases, and the mean value was 133.5 µg/L ± 177.9. The immunohistochemical examination using an anti-tryptase antibody on samples from the lungs, pharynx/larynx, and skin site of medication injection showed that all cases (100%) were strongly immunopositive for anti-tryptase antibody staining on lung samples; three cases (30%) were strongly immunopositive for anti-tryptase antibody staining on pharyngeal/laryngeal samples; and eight cases (80%) were strongly immunopositive for anti-tryptase antibody staining on skin samples. We conclude that a typical clinical history, blood tryptase level >40 µg/L, and strongly positive anti-tryptase antibody staining in the immunohistochemical investigation may represent reliable parameters in the determination of anaphylactic death with the accuracy needed for forensic purposes.

Evaluation of methods for the rapid identification of Neisseria gonorrhoeae in a routine clinical laboratory

1976 ◽  
Vol 4 (1) ◽  
pp. 19-21
Author(s):  
H M Pollock
Keyword(s):  
Neisseria Gonorrhoeae ◽  
Clinical Laboratory ◽  
Fluorescent Antibody ◽  
Rapid Identification ◽  
Reliable Identification ◽  
Initial Trial ◽  
Carbohydrate Degradation ◽  
Routine Clinical Laboratory ◽  
Evaluation Of Methods ◽  
Produce Acid

Of 78 isolates of Neisseria gonorrhoeae, 21 failed to grow and produce acid in unsupplemented cystine-Trypticase agar (CTA); whereas positive reactions were obtained by using serum-supplemented CTA and fluorescent antibody (FA). An additional 290 strains of Neisseria were evaluated by FA and by a rapid carbohydrate degradation technique (RF). There was agreement between the two methods 92% of the time on the initial trial and 99% of the time with repeats on discrepancies. The RF and FA tests provided rapid and reliable identification of N. gonorrhoeae, alleviating the problems of CTA due to lack of growth and need for overnight incubation.

scholarly journals Proliferation through activation: hemophagocytic lymphohistiocytosis in hematologic malignancy

2017 ◽  
Vol 1 (12) ◽  
pp. 779-791 ◽  
Author(s):  
Eric J. Vick ◽  
Kruti Patel ◽  
Philippe Prouet ◽  
Mike G. Martin
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Hematologic Malignancy ◽  
Lymphocytic Infiltration ◽  
Hematologic Malignancies ◽  
Interferon Γ ◽  
Host Immune System ◽  
T Helper ◽  
T Helper 1 ◽  
Factor Α ◽  
Necrosis Factor

AbstractHemophagocytic lymphohistiocytosis (HLH) is a syndrome of cytokine-driven immune activation. Cardinal features include fever, hemophagocytosis, hepatosplenomegaly, lymphocytic infiltration, and hypercytokinemia that result in multisystem organ dysfunction and failure. Familial HLH is genetically driven, whereas secondary HLH (SHL) is caused by drugs, autoimmune disease, infection, or cancer. SHL is associated with worse outcomes, with a median overall survival typically of less than 1 year. This reflects difficulty in both diagnostic accuracy and in establishing reliable treatments, especially in cases of malignancy-induced SHL, which have significantly worse outcomes. Malignancy-induced HLH is seen almost exclusively with hematologic malignancies, constituting 97% of cases in the literature over the past 2 years. In these situations, the native immune response driven by CD8 T cells produces an overabundance of T helper 1 cytokines, notably interferon-γ, tumor necrosis factor-α, and interleukin-6, which establish a positive feedback loop of inflammation, enhancing replication of hematologic malignancies while leaving the host immune system in disarray. In this paper, we present 2 case studies of secondary HLH driven by HM, followed by a review of the literature discussing the cytokines driving HLH, diagnostic criteria, and current treatments used or undergoing investigation.

scholarly journals Hemophagocytic Lymphohistiocytosis Secondary to Disseminated Histoplasmosis in Rheumatologic Disease

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Yael Kusne ◽  
Michael Christiansen ◽  
Christopher Conley ◽  
Juan Gea-Banacloche ◽  
Ayan Sen
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Pediatric Patients ◽  
Histoplasma Capsulatum ◽  
Hematologic Malignancies ◽  
Rapid Diagnosis ◽  
Rheumatologic Disease ◽  
Disseminated Histoplasmosis ◽  
Serious Infections ◽  
Time To Diagnosis ◽  
Prompt Diagnosis

Background. Hemophagocytic lymphohistiocytosis (HLH) was originally described in pediatric patients presenting with fever, hepatosplenomegaly, and blood cell abnormalities. Later, HLH was recognized to occur in adults, often associated with hematologic malignancies or serious infections. Conclusion. Patients presenting with HLH are critically ill, and rapid diagnosis is key. In adults, the search for the trigger must begin promptly as time to diagnosis effects survival. The underlying trigger in our patients was Histoplasma capsulatum infection, which is rare in the southwestern United States. Prompt diagnosis led to recovery in one patient, while the other did not survive.

Rapid identification of and assessment of damage by inhaled volatile substances in the clinical laboratory

1972 ◽  
Vol 5 (1-4) ◽  
pp. 222-231 ◽  
Author(s):  
W.C. Griffiths ◽  
M. Lipsky ◽  
A. Rosner ◽  
H.F. Martin
Keyword(s):  
Clinical Laboratory ◽  
Rapid Identification ◽  
Volatile Substances

scholarly journals A new multiplex PCR-based reverse line-blot hybridization (mPCR/RLB) assay for rapid staphylococcal cassette chromosome mec (SCCmec) typing

2009 ◽  
Vol 58 (8) ◽  
pp. 1045-1057 ◽  
Author(s):  
Lin Cai ◽  
Fanrong Kong ◽  
Qinning Wang ◽  
Huiping Wang ◽  
Meng Xiao ◽  
...  
Keyword(s):  
Multiplex Pcr ◽  
Clinical Laboratory ◽  
Blot Hybridization ◽  
Rapid Identification ◽  
Reverse Line Blot ◽  
Discriminatory Power ◽  
Hybridization Assay ◽  
Coagulase Negative Staphylococci ◽  
Primer Sets ◽  
Mrsa Strains

The aim of this study was to develop a new discriminatory method for MRSA SCCmec typing based on multiplex PCR-based reverse line-blot hybridization (mPCR/RLB) assay to enable rapid identification and classification of MRSA SCCmec types in a clinical laboratory. Forty-five primer sets and 49 probes were designed and tested in uniplex PCR (uPCR) and mPCR/RLB. Probes were compared in silico to 14 whole-genome sequences and 18 partial SCCmec gene sequences of Staphylococcus aureus and complete genome and partial SCCmec genes of seven non-MRSA strains, including meticillin-susceptible S. aureus and meticillin-resistant coagulase-negative staphylococci. The method was tested on a set of 42 well-characterized reference MRSA strains. It identified all five epidemiologically relevant SCCmec types and 26 subtypes, including established and new subtypes of SCCmec III, IV (eight subtypes each) and V (three subtypes). The discriminatory power of mPCR/RLB SCCmec typing was similar to that of MLST and spa typing (Simpson indices of diversity of 0.916, 0.926 and 0.882, respectively; differences not statistically significant). The application of mPCR/RLB hybridization assay to MRSA SCCmec typing can improve the specificity, discriminatory power and throughput of the typing procedure. The detection of up to 43 mPCR products in a single hybridization assay transforms MRSA SCCmec typing from passive epidemiological library typing into a potential tool for near-real-time infection control surveillance and tracking of MRSA transmission in hospitals.

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