Hepatobiliary Dysfunction and Disseminated Intravascular Coagulation Increase Risk of Mortality in Pediatric Hemophagocytic Lymphohistiocytosis*

2018 ◽  
Vol 19 (10) ◽  
pp. e522-e530 ◽  
Author(s):  
Jordana Goldman ◽  
Moreshwar S. Desai ◽  
Kenneth L. McClain ◽  
M. Hossein Tcharmtchi ◽  
Curtis E. Kennedy ◽  
...  
Keyword(s):  
Disseminated Intravascular Coagulation ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Intravascular Coagulation ◽  
Risk Of Mortality ◽  
Increase Risk

scholarly journals ALK-Negative Anaplastic Large Cell Lymphoma Presenting as Disseminated Intravascular Coagulation and Hemophagocytic Lymphohistiocytosis: A Potentially Fatal Presentation

2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
Uroosa Ibrahim ◽  
Amina Saqib ◽  
Maryam Rehan ◽  
Jean Paul Atallah
Keyword(s):  
Disseminated Intravascular Coagulation ◽  
Anaplastic Large Cell Lymphoma ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Lymph Node Biopsy ◽  
Intravascular Coagulation ◽  
Primary Disorder ◽  
Life Threatening ◽  
Large Cell Lymphoma

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder that can be familial in etiology or a result of infections, malignancy, and autoimmune or inflammatory disorders. Disseminated intravascular coagulation (DIC) is common in patients admitted to intensive care units and can confound and delay the diagnosis of HLH. We present a case of a 69-year-old female who presented with dyspnea and malaise. Her condition declined rapidly with laboratory parameters consistent with DIC. In addition, she had a ferritin of 32,522 ng/mL, low haptoglobin, and elevated LDH, and bone marrow biopsy showed hemophagocytic lymphohistiocytes. She was started on HLH-directed therapy, and later, a diagnosis of ALK-negative anaplastic large cell lymphoma was made on an excisional inguinal lymph node biopsy specimen. Our case emphasizes the importance of prompt recognition, diagnosis, and treatment of HLH while workup for a primary disorder is still being pursued.

scholarly journals Linfohistiocitose Hemofagocítica Secundária a Infeção por Epstein-Barr: Raridade e Gravidade num Adulto Imunocompetente

2019 ◽  
Vol 32 (1) ◽  
pp. 78
Author(s):  
Andreia Sofia Costa ◽  
Anusca Paixão ◽  
Henrique Santos ◽  
Fernando Salvador
Keyword(s):  
Disseminated Intravascular Coagulation ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Epstein Barr Virus ◽  
Clinical Case ◽  
Specific Treatment ◽  
Supportive Therapy ◽  
Intravascular Coagulation ◽  
Barr Virus ◽  
Life Threatening ◽  
Epstein Barr

Hemophagocytic lymphohistiocytosis is a rare, aggressive and life-threatening syndrome, characterized by an excessive immune activation. It is triggered by multiple stimuli, with infections having an important role. The most common infectious trigger is viral infection, particularly by Epstein-Barr virus. Coagulation disorders are common in hemophagocytic lymphohistiocytosis and disseminated intravascular coagulation can be present in severe cases. We report a clinical case of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis, complicated with disseminated intravascular coagulation which evolved favorably with only supportive therapy and without specific treatment.

scholarly journals Monitoring coagulation-fibrinolysis activation prompted timely diagnosis of hemophagocytic lymphohistiocytosis-related disseminated intravascular coagulation

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Liqin Ling ◽  
Xunbei Huang ◽  
Chaonan Liu ◽  
Juan Liao ◽  
Jing Zhou
Keyword(s):  
Disseminated Intravascular Coagulation ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Early Stage ◽  
Score System ◽  
Intravascular Coagulation ◽  
Timely Diagnosis ◽  
Organ Failures ◽  
Diagnostic Work ◽  
New Biomarkers ◽  
Work Up

Abstract Background Timely diagnosis of disseminated intravascular coagulation (DIC) in hemophagocytic lymphohistiocytosis (HLH) patients is crucial but challenging, as HLH interferes with the results of the laboratory tests included in the DIC score system. Case presentation Here, we reported a case of lymphoma-associated HLH, in which coagulation-fibrinolysis activation /inhibition markers (TAT, tPAIC, and PIC), prompted timely diagnosis of early stage DIC (initial phase of microvascular thrombosis, yet non-overt), prior to the development of organ failures and/or bleedings. Conclusions This report highlights the importance of the implementation of new biomarkers (such as TAT, tPAIC, and PIC), into the diagnostic work-up for coagulation disorders. These biomarkers are directly suggestive of microthrombus formation, therefore they can be of paramount importance in diagnosing DIC with complicated etiologies, such as hematological diseases-related DIC.

Disseminated Intravascular Coagulation: Clinical Diagnosis and Management

2019 ◽  
pp. 337-344
Author(s):  
Prakash Vishnu ◽  
Sikander Ailawadhi
Keyword(s):  
Innate Immunity ◽  
Severe Sepsis ◽  
Critical Illness ◽  
Clinical Diagnosis ◽  
Disseminated Intravascular Coagulation ◽  
Coagulation Activation ◽  
Intravascular Coagulation ◽  
Risk Of Mortality ◽  
Life Threatening ◽  
Inflammatory System

Disseminated intravascular coagulation (DIC) is a phenomenon with the potential for causing thrombosis and bleeding. DIC, typically occurring in patients with critical illness, can manifest as an acute, life-threatening emergency or as a chronic, subclinical process depending on the influence of morbidity from the underlying cause. The presence of DIC increases the risk of mortality by twofold in patients with trauma and severe sepsis and is an independent predictor of mortality. The pathogenesis of DIC is not only related to abnormal coagulation activation and platelet consumption but also involves multiple mechanisms of the inflammatory system and innate immunity.

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