Disseminated Intravascular Coagulation Associated with Large Deletion of Immunoglobulin Heavy Chain

Although the majority of monogenic defects underlying primary immunodeficiency are microlesions, large lesions like large deletions are rare and constitute less than 10% of these patients. The immunoglobulin heavy chain (IGH) locus is one of the common regions for such genetic alterations. This study describes a rare case of autosomal recessive agammaglobulinemia with a homozygous large deletion in chromosome 14q32.33 (106067756-106237742) immunoglobulin heavy chain clusters with an unusual and severe skin infection and disseminated intravascular coagulopathy.

Convalescent plasma does not provide adequate replacement of C1-Inhibitor and complements in COVID-19 patients

The rationale of Convalescent Plasma (CP) is to provide passive immunity to acutely ill COVID-19 patients. However, there are other pathologies of COVID disease that may be alleviated by CP. COVID-19 coagulopathy has been hypothesized to be a form of disseminated intravascular coagulopathy, a type of thrombotic microangiopathy. Complement activation has also been implicated in COVID-19 coagulopathy. An alternative hypothesis for additional benefits of CP is replacement of inhibitors of complements. C1-esterase inhibitor (C1-INH) is a major regulator of complement activation. We hypothesized that COVID-19 patients have decreased C1-INH and that CP transfusion would restore intravascular C1-INH and complement levels. We studied serial C1-INH and complement levels in COVID-19 patients before and after CP transfusion (200 mL) and their association with overall mortality. Methods: We identified COVID-19 patients (n=91) that received CP within the first 72 hours after admission. We collected serum and/or plasma samples at day prior and post-day 1, 3, and 10. C1 inhibitor, C3 and C4 were tested in these samples as well as in the respective CP unit given to each patient. Results: C1-INH levels day before transfusion were increased in COVID-19 patients (201.5% +/- 53%) in comparison to CP (93.2% +/- 26.2%). C1-INH transiently increased post CP transfusion and remained relatively high through day 10. No statistical difference was observed in C1-INH between survivors (n=53) and non-survivors (n=39) at any time point before or after transfusion. C3 was higher in COVID-19 patients in comparison to CP (161.5 +/-47.0 vs. 89.6 +/-15.3 mg/dL). However, C3 levels were significantly lower in non-survivors compared to survivors the day before transfusion (131.9 +/- 38.0 vs. 180.9 +/- 45.1 mg/dL, p=2.8E-06). Following transfusion, C3 levels decreased and remained steady afterwards; at all subsequent time points C3 levels were significantly lower in non-survivors compared to survivors (post-day 1: 130.6+/- 33.0 vs. 158.5 +/- 51.5 mg/dL, p=0.006; post-day 3: 116.6+/-46.5 vs. 146.2+/-42.8 mg/dL, p=0.01; post-day 10: 120.8+/-40.9 vs. 150.3+/- 45.9 mg/dL, p=0.03). C4 levels trended lower in non-survivors compared to survivors the day before transfusion (30.8+/- 15.3 vs. 37.9 +/- 16.7 mg/dL, p=0.08). The day following CP, there was a significant decrease in C4 across the entire cohort (35.1+/- 16.4 vs. 27.9+/- 18.3 mg/dL, p=0.01); subsequent levels remained steady. In conclusion, a single CP transfusion does not appear to restore C1-INH, C3 and C4 levels in hospitalized COVID-19 patients. CP transfusion is associated with a transient increase in C1-INH and decreasing C3 and C4 levels. Contrary to our hypothesis, C1-INH levels are increased in COVID-19 patients. The relationship between C1-INH and complements in COVID-19 remains to be fully elucidated. Prospective studies are needed to further delineate these relationships especially in the context of ongoing clinical trials of recombinant C1-INH in COVID-19 patients.

Massive late postoperative bleeding after abdominal surgery in a haematologic patient with postoperative CoV-2 infection

The role of viral infection in extrapulmonary postoperative complications in CoV-2 patients is still debated. Perioperative bleeding is rare compared with thrombotic events, but can be related to a haemorrhagic CoV-2-associated disseminated intravascular coagulopathy-like syndrome.

Paraneoplastic Phenomena of Disseminated Intravascular Coagulopathy in Hepatic Angiosarcoma – Rare, Challenging and Fatal. Case Report and Literature Review

Background. Hepatic angiosarcoma is an uncommon, malignant, primary liver tumor, comprising 2% of liver cancers and accounting for < 1% of all sarcomas. Patients usually present with nonspecific symptoms, such as fatigue, weight loss, right upper quadrant pain, anemia, which leads to late diagnosis of an advanced stage tumor. The median life expectancy after the diagnosis of hepatic angiosarcoma is about 6 months, with only 3% of patients surviving more than 2 years. Liver failure and hemoperitoneum are the leading causes of death in patients with liver angiosarcoma. In rarer cases, it might cause paraneoplastic syndromes such as disseminated intravascular coagulopathy. The treatment of angiosarcomas is complicated as there are no established and effective treatment guidelines due to the tumor’s low frequency and aggressive nature.Case summary. We present the case of a 68-year old woman who was admitted to the hospital due to fatigueand severe anemia (hemoglobin 65 g/l). Laboratory results also revealed high-grade thrombocytopenia(8 × 109/l). The abdominal ultrasound and computed tomography scan showed multiple lesions throughout with hepatic angiosarcoma. The treatment with first-line chemotherapy (doxorubicin) was initiated despiteongoing paraneoplastic syndrome – disseminative intravascular coagulopathy. However, the disease was terminal, and the patient died 2 months since diagnosed.Conclusions. Hepatic angiosarcoma is a rare and terminal tumor. Therefore, knowledge about its manifestations and effective treatment methods is lacking. Disseminative intravascular coagulopathy is a unique clinical characteristic of angiosarcoma seen in a subset of patients.

Disseminated Intravascular Coagulopathy in Critically Ill Patients in Amman, Jordan

Background: The disseminated intravascular coagulation (DIC) is under-recognized in critically ill patients. The International Society of Thrombosis and Haemostasis (ISTH; DIC) provides a useful scoring system for accurate DIC identification. The study investigated the period prevalence of ISTH DIC from 2015 to 2017 in critically ill patients. Methods: In this multi-center, retrospective observational study, we included all patients identified with a DIC code or medically diagnosed with DIC during all admissions. Based on ISTH DIC scores ≥ 5, patients were classified with overt DIC. Results: A total of 220 patients were included in this study. The period prevalence of DIC was 4.45%. The point prevalence of DIC has increased from 3.49% to 5.58% from 2015 to 2017 (27.7% female; median age 61.6 years). Based on the ISTH-Overt DIC criteria, 45.2% of the sample had sepsis. Overt DIC patients had significantly lower baseline hemoglobin (HB; t = 2.137, df = 193, p = 0.034), platelet count ( t = 3.591, df = 193, p < 0.001) and elevated serum creatinine level ( M = 2.1, SD = 1.5, t = 2.203, df = 193, p = 0.029) compared to non–Overt DIC. There was a statistically significant elevation in FDPs among Overt DIC compared to non–Overt DIC (χ2 = 30.381, df = 1, p < 0.001). Overt DIC patients had significantly prolonged PT ( U = 2,298, z = 5.7, p < 0.001), PTT ( U = 2,334, z = 2.0, p = 0.045) and INR ( U = 2,541, z = 5.1, p < 0.001) compared to those with non–Overt DIC. Conclusion: The ISTH overt-DIC score can be used in critically ill patients regardless of the underlying disease. Efforts are required to predict and identify overt DIC using a valid scoring system on admission and follow-up of adult patients admitted to ICU.

Heat stroke in strenuous exercises: a clinical case

According to the Association of International Marathons and Distance Races, more than 800 marathons are held annually in the world, but excessive physical activity during marathon races involves significant health risks. We would like to bring to your attention a case history of heat stroke in a young athlete during the half-marathon running, which manifested itself by heat damage to vascular endothelium of almost all organs, rapid development of disseminated intravascular coagulopathy, consumption coagulopathy, hyperfibrinolysis, multiple organ dysfunction syndrome involving the heart, lungs, liver, kidneys, gastrointestinal tract, brain, and bone marrow, which ended in death.

Uterus Preservation Versus Caesarean Hysterectomy in Management of Placenta Accreta Spectrum: A Multicentre International Study

Objective: To compare peripartum outcomes of uterus preserving procedures to caesarean hysterectomy in women with placenta accreta spectrum (PAS), and to identify risk factors associated with adverse maternal outcomes. Design: Retrospective study (ClinicalTrials.gov identifier: NCT04384510) Setting:11 tertiary centres from 9 countries Population or Sample: women with of PAS who were managed in participating centres between January 1st, 2010 and December 31st, 2019. Women who had confirmed diagnosis with PAS with adequate documentation and follow-up, were considered eligible. Main Outcome Measures: Primary outcome was massive PAS-associated perioperative blood loss (intraoperative blood loss ≥ 2500 ml, bleeding associated massive transfusion protocol, or complicated by disseminated intravascular coagulopathy). Results: Out of 797 women, 727 were eligible for the study. Five hundred ninety-two (81.43%) women were managed by uterus preserving procedures versus 135 (18.56%) who underwent caesarean hysterectomy. After adjustment for significant or close-to-significance variables, type of management was not associated with higher risk of massive blood loss (aOR 1.71, 95% CI 0.78 - 3.81). Other factors that were significantly associated with higher risk of massive PAS-associated blood loss included body mass index, preoperative haemoglobin, centrally located placenta, diffuse placental invasion, parametrial invasion, and intrauterine foetal death. Conclusions: In the presence of sufficient experience, uterus preserving procedures may not be associated with higher risk of massive blood loss compared to caesarean hysterectomy. Funding: none