Hematology Flashcards
What is the primary cause of Thrombotic Thrombocytopenic Purpura (TTP)?
Platelet adhesion and activation, resulting in microvascular ischemia and microangiopathic hemolytic anemia.
What is the role of ADAMTS-13 in TTP?
Inherited deficiency of ADAMTS-13 can lead to TTP, but not all individuals with this deficiency develop the condition.
Which drugs are implicated as causative agents in TTP?
Ticlopidine and possibly clopidogrel can induce antibody formation causing TTP. Other drugs include mitomycin C, cyclosporine, and quinine, causing endothelial toxicity.
How can TTP be differentiated from disseminated intravascular coagulation (DIC)?
TTP causes microangiopathic hemolytic anemia (MAHA) without a predominant coagulopathy, while DIC has a significant coagulopathy.
What is the mortality rate for untreated TTP?
85% to 100%.
What is the primary treatment for TTP?
Plasma exchange.
What adjunctive treatment can be used in TTP?
Glucocorticoids.
What recent research suggests about rituximab in TTP treatment?
Rituximab may be useful in primary treatment of TTP, but relapse is common.
What are the two types of hemophilia associated with factor deficiencies?
Hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency).
What is the prevalence of hemophilia A compared to hemophilia B?
Hemophilia A accounts for about 80% of hemophilia cases.
What factor activity levels indicate severe hemophilia?
Less than 1%.
What are common clinical manifestations of moderate and severe hemophilia?
Bleeding into joints, soft tissues, and muscles after minimal trauma or spontaneously.
What is the goal factor activity level for treating large hematomas or deep muscle bleeds?
Raise the activity level to 50%.
What is the role of DDAVP in hemophilia A treatment?
It can be given as adjunctive therapy for acute bleeding episodes to cause a transient rise in factor VIII levels.
What are the recommended factor activity levels following treatment for milder bleeds?
30% to 50% initially, maintaining levels between 15% and 25% for 2 to 3 days.
What is the life expectancy for patients with hemophilia given recent therapy advancements?
About 65 years.
What treatment is recommended for individuals with baseline factor activity levels of less than 1%?
Primary prophylaxis to prevent spontaneous bleeding.
Fill in the blank: Hemophilia is primarily treated with _______.
[factor concentrates].
True or False: Antifibrinolytic drugs like tranexamic acid are helpful in promoting hemostasis for mucosal bleeding.
True.
What is a significant risk factor for hemophilia patients due to early treatment methods?
Bloodborne pathogens such as HIV and hepatitis C.
What is the recommended treatment duration for an individual requiring factor concentrates?
A period of 7 days or more may be required.
What are the characteristics of red blood cells (RBCs) in patients with sickle cell disease?
Less pliable and more sticky than normal RBCs
What can precipitate a vaso-occlusive crisis in sickle cell disease?
Infection, fever, excessive exercise, anxiety, abrupt changes in temperature, hypoxia, hypertonic dyes
What does a peripheral blood smear show in sickle cell disease?
Elongated, crescent-shaped RBCs
