scholarly journals Chronic lymphocytic leukemia-associated paraneoplastic pemphigus: potential cause and therapeutic strategies

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Lei Cao ◽  
Fei Wang ◽  
Xin-Yi Du ◽  
Hua-Yuan Zhu ◽  
Li Wang ◽  
...  
Keyword(s):  
Median Overall Survival ◽  
Standard Treatment ◽  
Secondary Infection ◽  
Fresh Frozen Plasma ◽  
Lymphocytic Leukemia ◽  
Short Term ◽  
Paraneoplastic Pemphigus ◽  
Autoimmune Syndrome ◽  
Fresh Frozen ◽  
Frozen Plasma

Abstract Paraneoplastic pemphigus (PNP) is a severe autoimmune syndrome commonly triggered by neoplasms. The prognosis of CLL-associated PNP is dismal due to its refractory course and secondary infection and no standard treatment was recommended. We retrospectively reported six CLL with PNP cases from 842 cases of CLL including diagnosis, treatment and prognosis. The median time between the initial of CLL to PNP was 36 months while the median overall survival from the diagnosis of PNP was 26 months. And three cases died of lung infection while 5 developed pulmonary symptoms. And 5 cases received fludarabine-based chemotherapy before developing PNP, which suggesting fludarabine was one of potential causes of PNP. For the treatment, five patients were rescued by combined regimens including rituximab, methylprednisolone, immunoglobulin, fresh frozen plasma and the last received ibrutinib combined with short-term prednisone. Fludarabine-based regimen may be one of the potential causes of PNP. The combined regimen might shed a new light, while ibrutinib is a promising drug for CLL with PNP, but needs much more evidence. PNP should be carefully treated to guide early diagnosis and intervention for a better prognosis.

Enhancing the Action of Rituximab in Chronic Lymphocytic Leukemia by Adding Fresh Frozen Plasma

2009 ◽  
Vol 1173 (1) ◽  
pp. 865-873 ◽  
Author(s):  
Abraham Klepfish ◽  
Lugassy Gilles ◽  
Kotsianidis Ioannis ◽  
Rachmilewitz Eliezer ◽  
Schattner Ami
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Fresh Frozen Plasma ◽  
Lymphocytic Leukemia ◽  
Fresh Frozen ◽  
Frozen Plasma

scholarly journals Practical management for urgent reversal of oral anticoagulation in patients with acute intracerebral haemorrhage

2011 ◽  
Vol 2 (1S) ◽  
pp. 93
Author(s):  
Davide Imberti
Keyword(s):  
Vitamin K ◽  
Intracerebral Haemorrhage ◽  
Gold Standard ◽  
Prothrombin Complex Concentrate ◽  
Standard Treatment ◽  
Oral Anticoagulant Therapy ◽  
Fresh Frozen Plasma ◽  
Emergency Intervention ◽  
Fresh Frozen ◽  
Frozen Plasma

In case of intracerebral haemorrhage (ICH) during oral anticoagulant therapy (OAT) it is mandatory to obtain the fast and complete normalisation of haemostasis, in order to minimise the risk of haematoma enlargement. Furthermore, if neurosurgery is requested, the immediate correction of haemostatic balance allows the execution of emergency intervention, thus reducing the risk of intra- and post-surgical haemorrhagic complications. Currently prothrombin complex concentrate (PCC) in combination with vitamin K represents the gold standard treatment for patients with ICH during OAT. This treatment should be preferred to the administration of fresh frozen plasma (FFP) in order to guarantee a fast and almost immediate normalisation of blood coagulation.

scholarly journals Practical management for urgent reversal of oral anticoagulation in patients with acute intracerebral haemorrhage

2011 ◽  
Vol 2 (1S) ◽  
pp. 93-98
Author(s):  
Davide Imberti
Keyword(s):  
Vitamin K ◽  
Intracerebral Haemorrhage ◽  
Gold Standard ◽  
Prothrombin Complex Concentrate ◽  
Standard Treatment ◽  
Oral Anticoagulant Therapy ◽  
Fresh Frozen Plasma ◽  
Emergency Intervention ◽  
Fresh Frozen ◽  
Frozen Plasma

In case of intracerebral haemorrhage (ICH) during oral anticoagulant therapy (OAT) it is mandatory to obtain the fast and complete normalisation of haemostasis, in order to minimise the risk of haematoma enlargement. Furthermore, if neurosurgery is requested, the immediate correction of haemostatic balance allows the execution of emergency intervention, thus reducing the risk of intra- and post-surgical haemorrhagic complications. Currently prothrombin complex concentrate (PCC) in combination with vitamin K represents the gold standard treatment for patients with ICH during OAT. This treatment should be preferred to the administration of fresh frozen plasma (FFP) in order to guarantee a fast and almost immediate normalisation of blood coagulation.

Short-term prophylaxis for children and adolescents with hereditary angioedema

2021 ◽  
Vol 42 (3) ◽  
pp. 205-213
Author(s):  
Oyindamola Ajewole ◽  
Mosopefoluwa Lanlokun ◽  
Stevenson Dimanche ◽  
Timothy Craig
Keyword(s):  
Tranexamic Acid ◽  
Hereditary Angioedema ◽  
Upper Airway ◽  
Fresh Frozen Plasma ◽  
Published Data ◽  
C1 Inhibitor ◽  
Short Term ◽  
Fresh Frozen ◽  
Short Term Prophylaxis ◽  
Frozen Plasma

Background: Hereditary Angioedema (HAE) is a rare, autosomal dominant, life threatening disease, secondary to the deficiency of C1-inhibitor, dysfunction of C1-inhibitor or inadequate control of the contact pathway. Presentation includes recurrent swelling of the skin, upper airway and the abdomen. Trauma can precipitate attacks, which in the airway can lead to asphyxia. For this reason, short term prophylaxis (STP) may be indicated before medical, surgical and dental procedures. The goal of the manuscript is to review short term prophylaxis for children of all ages. Methods: We searched the following search words: children, pediatric, adolescent, plasma derived C1-inhibitor, recombinant C1-inhibitor, surgery, medical procedures, prophylaxis, dental, Hereditary Angioedema, tranexamic acid, androgens, fresh frozen plasma, short term prophylaxis, lanadelumab, subcutaneous C1-inhibitor in Google Scholar and in PubMed to develop our results. Results: STP should be discussed at every visit. Plans should be individualized based upon the procedure, therapies available and shared decision making with patient/parent. For high risk procedures plasma derived C1-inhibitor should be used at 20 units/kg just prior to the procedure. Alternative agents for STP include recombinant C1-inhibitor, fresh frozen plasma, androgens, or tranexamic acid. In all cases, with or without the use of STP, 2 doses of on-demand therapy should be available in case of an attack. Conclusion: Herein, we review the published data on STP for pediatric patients with HAE and discuss first-line options, and off label use of medications, as well as review the guidelines pertaining to short term prophylaxis.

Enhancing the action of rituximab by adding fresh frozen plasma for the treatment of fludarabine refractory chronic lymphocytic leukemia

2010 ◽  
Vol 128 (9) ◽  
pp. 2192-2201 ◽  
Author(s):  
Wei Xu ◽  
Kou-Rong Miao ◽  
Dan-Xia Zhu ◽  
Cheng Fang ◽  
Hua-Yuan Zhu ◽  
...  
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Fresh Frozen Plasma ◽  
Lymphocytic Leukemia ◽  
Fresh Frozen ◽  
Frozen Plasma

Das Smith-Lemli-Opitz-Syndrom

2005 ◽  
Vol 5 (04) ◽  
pp. 178-182
Author(s):  
Wieland Kiess ◽  
Manuela Schulz ◽  
Sabine Liebermann ◽  
Roland Pfäffle ◽  
Peter Bührdel ◽  
...  
Keyword(s):  
Fresh Frozen Plasma ◽  
Therapeutische Möglichkeiten ◽  
Fresh Frozen ◽  
Frozen Plasma

ZusammenfassungDas Smith-Lemli-Opitz-Syndrom wird durch einen Defekt des letzten Schrittes der Cholesterolbiosynthese, den Mangel an 7-Dehydrocholesterolreduktase, verursacht. Die Akkumulation der Metaboliten 7-Dehydrocholesterol und 8-Dehydrocholesterol, die die wichtigsten biochemischen Marker für die Diagnose der Erkrankung darstellen, sowie der Mangel an Cholesterol können zu multiplen kongenitalen Anomalien führen. Die Ursache des Enzymmangels sind Mutationen innerhalb des DHCR7-Gens, welches auf Chromosom 11q13 lokalisiert ist. Therapeutische Möglichkeiten bestehen in der Gabe von Cholesterol und im Notfall Fresh Frozen Plasma (FFP); der therapeutische Nutzen von Statinen befindet sich zurzeit in der klinischen Erprobung.

The Recovery of Factor VIII from Fresh-Frozen, Indated and Outdated Human Plasma

1976 ◽  
Vol 36 (01) ◽  
pp. 071-077 ◽  
Author(s):  
Daniel E. Whitman ◽  
Mary Ellen Switzer ◽  
Patrick A. McKee
Keyword(s):  
Factor Viii ◽  
Polyacrylamide Gel Electrophoresis ◽  
Sodium Dodecyl ◽  
The United States ◽  
Fresh Frozen Plasma ◽  
Red Cross ◽  
Random Samples ◽  
Fresh Frozen ◽  
Factor Viii Concentrates ◽  
Frozen Plasma

SummaryThe availability of factor VIII concentrates is frequently a limitation in the management of classical hemophilia. Such concentrates are prepared from fresh or fresh-frozen plasma. A significant volume of plasma in the United States becomes “indated”, i. e., in contact with red blood cells for 24 hours at 4°, and is therefore not used to prepare factor VIII concentrates. To evaluate this possible resource, partially purified factor VIII was prepared from random samples of fresh-frozen, indated and outdated plasma. The yield of factor VIII protein and procoagulant activity from indated plasma was about the same as that from fresh-frozen plasma. The yield from outdated plasma was substantially less. After further purification, factor VIII from the three sources gave a single subunit band when reduced and analyzed by sodium dodecyl sulfate polyacrylamide gel electrophoresis. These results indicate that the approximately 287,000 liters of indated plasma processed annually by the American National Red Cross (ANRC) could be used to prepare factor VIII concentrates of good quality. This resource alone could quadruple the supply of factor VIII available for therapy.

Haemophilia in a Female

1971 ◽  
Vol 26 (02) ◽  
pp. 205-210
Author(s):  
J. A McBride ◽  
J Hunter ◽  
Elizabeth Pearse ◽  
Yvette Sultan ◽  
J. P Caen
Keyword(s):  
Fresh Frozen Plasma ◽  
Plasma Fibrinogen ◽  
Fresh Frozen ◽  
Frozen Plasma

SummaryA case of haemophilia in a female is described together with the response of the patient’s level of antihaemophilic factor in the plasma following transfusion of fresh frozen plasma, fibrinogen and cryoprecipitate.

Severe Inherited “Homozygous” Protein C Deficiency in a Newborn Infant

1984 ◽  
Vol 52 (01) ◽  
pp. 053-056 ◽  
Author(s):  
A Estellés ◽  
I Garcia-Plaza ◽  
A Dasí ◽  
J Aznar ◽  
M Duart ◽  
...  
Keyword(s):  
Newborn Infant ◽  
Protein C ◽  
Skin Lesions ◽  
Fresh Frozen Plasma ◽  
Clinical Syndrome ◽  
Assay Method ◽  
Enzyme Linked Immunosorbent Assay ◽  
Protein C Deficiency ◽  
Fresh Frozen ◽  
Frozen Plasma

SummaryA relapsing clinical syndrome of skin lesions and disseminated intravascular coagulation (DIC) that showed remission with the infusion of fresh frozen plasma is described in a newborn infant with homozygous deficiency of protein C antigen.This patient presented since birth a recurrent clinical picture of DIC and ecchymotic skin lesions that resembled typical ecchymosis except for the fact that they showed immediate improvement with the administration of fresh frozen plasma. Using an enzyme linked immunosorbent assay method, the determination of protein C antigen levels in the patient, without ingestion of coumarin drugs, showed very low values (<1%).No other deficiencies in the vitamin-K-dependent factors or in anti thrombin III, antiplasmin, and plasminogen were found. Seven relatives of the infant had heterozygous deficiency in protein C antigen (values between 40-55%), without clinical history of venous thrombosis. The pedigree analysis of this family suggests an autosomal recessive pattern of inheritance for the clinical phenotype, although an autosomal dominant pattern has been postulated until now in other reported families.We conclude that our patient has a homozygous deficiency in protein C and this homozygous state may be compatible with survival beyond the neonatal period.

Comparison of a New Prototype for Rapid Thawing of Fresh Frozen Plasma with Two Commercially Available Thawing Devices

2019 ◽  
Author(s):  
A. Mandl ◽  
H. Rempel ◽  
S. Hackenbuchner ◽  
S. Rehberg ◽  
K. Leimkühler
Keyword(s):  
Fresh Frozen Plasma ◽  
Fresh Frozen ◽  
Frozen Plasma
Sign in / Sign up

Export Citation Format

Share Document