Congenital ADAMTS-13 deficiency presenting as life-threatening thrombosis during pregnancy

2021 ◽  
Vol 14 (8) ◽  
pp. e239901
Author(s):  
Faheema Hasan ◽  
Anshul Gupta ◽  
Dinesh Chandra ◽  
Soniya Nityanand
Keyword(s):  
Late Presentation ◽  
Fresh Frozen Plasma ◽  
Artery Thrombosis ◽  
Fresh Frozen ◽  
Life Threatening ◽  
A Disintegrin And Metalloproteinase ◽  
Thrombospondin Type 1 Repeats ◽  
Frozen Plasma ◽  
Plasma Infusions

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease characterised by thrombocytopenia, microangiopathic haemolytic anaemia and microvascular thrombosis. Congenital TTP accounting for less than 5% of all TTP cases can have a late presentation in adulthood mostly triggered by predisposing factors such as infection, pregnancy and inflammation. We present a case of a 23-year-old woman who presented to us in the postpartum period with mesenteric artery thrombosis with infarcts and later was diagnosed as a case of TTP based on congenital a disintegrin and metalloproteinase with thrombospondin type 1 repeats 13 (ADAMTS-13) deficiency detected on ADAMTS-13 levels and gene sequencing. She was successfully managed initially with therapeutic plasma exchanges and is now on prophylactic fortnightly fresh frozen plasma infusions at 15 mL/kg body weight and continues to be in remission.

Massive Transfusion

2018 ◽  
pp. 169-180
Author(s):  
Jay Berger
Keyword(s):  
Red Blood Cells ◽  
Blood Volume ◽  
Blood Cells ◽  
Massive Transfusion ◽  
Fresh Frozen Plasma ◽  
Metabolic Complications ◽  
Packed Red Blood Cells ◽  
Fresh Frozen ◽  
Life Threatening ◽  
Frozen Plasma

Massive transfusion is defined as transfusion of 3 units of packed red blood cells in less than 1 hour in an adult, replacement of more than 1 blood volume in 24 hours, or replacement of more than 50% of blood volume in 3 hours. Massive transfusion protocols are implemented in cases of life-threatening hemorrhage after trauma, during a surgical procedure, or during childbirth. These protocols are intended to minimize the adverse effects of hypovolemia, dilutional anemia, metabolic complications, and coagulopathy with early empiric replacement of blood products and transfusion of fresh frozen plasma, platelets, and packed red blood cells in a composition that approximates that of whole blood.

218 Impact of Fresh Frozen Plasma Infusions during Resuscitation in Thermally-Injured Patients

2018 ◽  
Vol 39 (suppl_1) ◽  
pp. S76-S76 ◽  
Author(s):  
V S Hoelscher ◽  
J A Harvin ◽  
B A Cotton ◽  
C E Wade ◽  
T F Huzar
Keyword(s):  
Fresh Frozen Plasma ◽  
Fresh Frozen ◽  
Injured Patients ◽  
Frozen Plasma ◽  
Plasma Infusions

scholarly journals Caution in diagnosing angioedema as anaphylaxis

2019 ◽  
Vol 12 (9) ◽  
pp. e230329 ◽  
Author(s):  
Shivam Patel ◽  
Roshan Mathew ◽  
Sanjeev Bhoi
Keyword(s):  
Ace Inhibitor ◽  
Fatal Outcome ◽  
Fresh Frozen Plasma ◽  
Definitive Treatment ◽  
Common Presentation ◽  
Good Outcome ◽  
Fresh Frozen ◽  
Life Threatening ◽  
The Common ◽  
Frozen Plasma

Angioedema is one of the commonest life-threatening conditions with good outcome timely definitive treatment. However, failure to recognise the common presentation of an uncommon bradykinin-mediated angioedema in time may lead to fatal outcome in the emergency department (ED). We report a case of a 79-year-old male patient who presented to ED with features of ACE inhibitor-induced angioedema which was identified and resuscitated by the emergency physician with use of fresh frozen plasma (FFP) leading to prompt recovery and good outcome.

Hereditary Angioedema: Management of Laryngeal Attacks

2011 ◽  
Vol 25 (6) ◽  
pp. 379-382 ◽  
Author(s):  
Sandra C. Christiansen ◽  
Bruce L. Zuraw
Keyword(s):  
Hereditary Angioedema ◽  
Treatment Options ◽  
The United States ◽  
Fresh Frozen Plasma ◽  
C1 Inhibitor ◽  
Acute Therapy ◽  
Fresh Frozen ◽  
Life Threatening ◽  
Frozen Plasma ◽  
Future Management

Background Hereditary angioedema (HAE) patients suffering from laryngeal attacks in the United States faced severely limited treatment options until 2008. These potentially life-threatening episodes occur in over one-half of the patients affected by HAE during their lifetimes. Acute therapy had been relegated to supportive care, intubation, and consideration of fresh frozen plasma (FFP)–-the latter with the potential for actually accelerating the speed and severity of the swelling. Methods In this article we will review the recently approved and emerging HAE treatments that have evolved from the recognition that bradykinin generation is the fundamental abnormality leading to attacks of angioedema. Results Acute therapy for laryngeal attacks will be discussed including purified plasma–derived C1 inhibitor (C1INH), recombinant C1INH, an inhibitor of plasma kallikrein (ecallantide), and a B2 receptor antagonist (icatibant). Prophylactic care has also been transformed from a reliance on attenuated androgens with their attendant side effects to C1INH replacement. Conclusion The arrival of these novel therapies promises to transform the future management of HAE.

scholarly journals Spontaneous Sublingual Haematoma in a 90-year Old Patient: A Complication of Direct Oral Anticoagulants

2020 ◽  
Vol 6 (2) ◽  
pp. 111-114
Author(s):  
Johannes Daniël Cnossen ◽  
Jeannette Fenna Schoonderbeek ◽  
Maaike Muller
Keyword(s):  
Rare Complication ◽  
Oral Anticoagulants ◽  
Direct Oral Anticoagulants ◽  
Upper Airway ◽  
Chronic Atrial Fibrillation ◽  
Fresh Frozen Plasma ◽  
Nasal Intubation ◽  
Fresh Frozen ◽  
Life Threatening ◽  
Frozen Plasma

AbstractSublingual haematoma is a rare complication of anticoagulants and can be life-threatening. As the number of prescribed anticoagulants is increasing, the incidence of complications of these drugs will continue to increase. A report of a sublingual haematoma in an elderly patient with chronic atrial fibrillation treated with edoxban (Lixiana ©, Daiichi Sankyo Europe GmbH, München, Germany) is reported. A 90-year male presented at the emergency department with an obstructed upper airway due to a sublingual haematoma. The patient received tranexamic acid, prothrombin complex, and fresh frozen plasma. After fiberoptic nasal intubation, the patient was monitored in the intensive care unit. After four days, the patient was extubated, and after six days, the swelling resolved completely. Complications of anticoagulants are rare but can be life-threatening. Recognition of an endangered airway and reversing the effects of the anticoagulant are essential. Surgical evacuation of the haematoma could be considered but is not necessary.

scholarly journals Congenital thrombotic thrombocytopenic purpura presenting in adulthood with recurrent cerebrovascular events

2019 ◽  
Vol 12 (10) ◽  
pp. e229481
Author(s):  
Emma Tenison ◽  
Ashar Asif ◽  
Mathew Sheridan
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Fresh Frozen Plasma ◽  
Vessel Occlusion ◽  
Thrombocytopenic Purpura ◽  
Antibody Titres ◽  
Fresh Frozen ◽  
Life Threatening ◽  
History Of ◽  
Congenital Thrombotic Thrombocytopenic Purpura ◽  
A Disintegrin And Metalloproteinase

Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare, life-threatening disease, characterised by episodes of microangiopathic haemolytic anaemia (MAHA), thrombocytopenia and small vessel thrombosis. We describe a case of cTTP first diagnosed at age 70 years in a female presenting with an acute ischaemic stroke and thrombocytopenia, in whom A Disintegrin And Metalloproteinase with a Thrombospondin type 1 Motif, member 13 (ADAMTS13) levels were <10%, suggestive of thrombotic thrombocytopaenic purpura (TTP). The patient underwent plasma exchange and started rituximab for presumed immune TTP; however, anti-ADAMTS13 antibody titres were negative on two occasions. This, together with a history of pregnancies complicated by presumed disseminated intravascular coagulation, and two previous episodes of sepsis with MAHA, prompted investigation for cTTP, which was confirmed by genetic testing. Despite treatment with infusions of solvent/detergent-treated, virus-inactivated fresh frozen plasma, she has re-presented with further neurological deficit, associated with new infarcts on imaging. cTTP has a varied phenotype which, as demonstrated in this case, can include large vessel occlusion.

Selective Immunoglobulin A Deficiency and Intestinal Nodular Lymphoid Hyperplasia: Correction of Diarrhea with Antibiotics and Plasma

PEDIATRICS ◽  
1968 ◽  
Vol 42 (5) ◽  
pp. 833-837
Author(s):  
Joyce D. Gryboski ◽  
Thomas W. Self ◽  
Arthur Clemett ◽  
Teodoro Herskovic
Keyword(s):  
Small Intestine ◽  
Abdominal Pain ◽  
Chronic Diarrhea ◽  
Immunoglobulin A ◽  
Lymphoid Hyperplasia ◽  
Fresh Frozen Plasma ◽  
Nodular Lymphoid Hyperplasia ◽  
Fresh Frozen ◽  
Frozen Plasma ◽  
Plasma Infusions

A 13-year-old boy with chronic diarrhea and abdominal pain since 6 months of age, and recurrent fevers since 12% years of age, was found to have deficiency of IgA and nodular lymphoid hyperplasia of the small intestine. This disorder was similar to that of "dysgammaglobulinemia and intestinal lymphoid hyperplasia" described in adults in 1966. An associated giardiasis was eliminated by atabrine therapy and a malabsorption syndrome, cleared after treatment with tetracycline. Diarrhea and abdominal pain were not alleviated until he was treated with monthly fresh frozen plasma infusions. It is to be emphasized that other forms of chronic diarrhea not related to immunoglobuun deficiencies do not respond to this type of therapy.

scholarly journals Thrombocytopenia‐associated multiorgan failure occurring in an infant at the onset of type 1 diabetes successfully treated with fresh frozen plasma

2016 ◽  
Vol 4 (7) ◽  
pp. 671-674 ◽  
Author(s):  
Revati Kumar ◽  
Brent McSharry ◽  
Peter Bradbeer ◽  
Esko Wiltshire ◽  
Craig Jefferies
Keyword(s):  
Type 1 Diabetes ◽  
Multiorgan Failure ◽  
Fresh Frozen Plasma ◽  
Fresh Frozen ◽  
Frozen Plasma
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