blood disorders Flashcards
hemophilia
- bleeding disorder involving abnormalities of coag factors
- causes spont bleeding
what are the types of hemophilia
- A
- B
- C
- categorized as mild, mod or severe
hemophilia A
- recessive X linked
- d/t deficient clotting factor VIII
hemophilia B
- recessive X linked
- d/t deficiency of clotting factor IX
hemophilia C
- autosomal genetic disorder
- lack of clotting factor XI
- affects Ashkenazi jews
- only mild sx
what is the most common type of hemophilia
- hemophilia A
clinical manifestations of hemophilia
- spont/ severe bleeding
- delayed bleeding after trauma or dental procedures
- bleeding from skin/ mucous membranes, epistaxis, ecchymosis
- hemoptysis
- hemarthrosis*
- severe ST bleeding
- compartment syndrome
- GIB
- ICH
diagnosis of hemophilia
- suspect if hx of easy bruising or bleeding
- get good family hx
- elevated PTT
- reduced factor VIII and IX
treatment of hemophilia
- ppx with factor VIII and IX
- promptly treat hemarthrosis
- recombinant vs plasma derived factors
- recominant factors have longer half lives, dont have ab production
- for acute bleed raise factor activity > 50%
complications of hemophilia tx
- transfusion transmitted infx (rare)
- dev inhibitory ab against factor VIII and IX
why is vit k needed for clotting
- cofactor to activate clotting factors within hepatic cells
- major part of coag pathways
where is vit k found
- green veggies
- oils
- produced by colonic bacteria
where is vit k absorbed
- terminal ileum into lymphatic sys, bile salts, and during normal fat absorption
causes of vit k deficiency
- decreased intake- elderly
- abnormal absorption
- liver disease*
- coumadin
- newborns naturally born with low levels
treatment for vit k deficiency
- vit K supplementation through diet or K1 (phytonadione)
- PO or IV
- infants get IM inj at birth
what is disseminated intravascular coagulation (DIC)
- consumption coagulopathy
- bleeding and clotting at the same time
- life threatening
- high mortality rates depending on cause
causes of DIC
- malignancy- breast ca
- trauma
- burns
- overwhelming sepsis and infection
- transfusions
- toxins
- obstetric causes
- liver disease
- prosthetic devices
pathophys of clotting in DIC
- tissue factor triggers coag cascade and thrombin production
- regulatory factors are consumed
- large amounts of thrombin generated*
- causes hypercoag state
pathophys of bleeding in DIC
- quantity of plasmin is increased
- causes significant quantities of fibrin degradation products ->bleeding
clinical presentation of DIC
- present with bleeding or clotting
- bleeding at any site- can be mild mucosal to severe
- purpura fulminans
- arterial thrombosis
- venous thrombosis
what is purpura fulminans
- cutaneous manifestation of DIC
- cutaneous intravasc thrombosis and hemorrhagic infarction of skin
- usu assoc with sepsis
work up for DIC
- get a good hx
- thrombocytopenia
- prolonged PT
- prolonged PTT
- increased thrombin production
treatment for DIC
- treat underlying cause
- ICU admission
- difficult balance between clotting and bleeding
- hemodynamic support
- platelet transfusion if severe thrombocytopenia
- FFP to correct coag factor def
- heparin if unstable thrombosis
- antithrombin infusion for anticoag properties
polycythemia vera
- chronic acquired myeloproliferative neoplasm
- d/t genetic mutation in JAK2
- abnormal hematopoietic production of bone marrow, esp RBCs
pathophys of polycythemia vera
- increased RBC mass and blood volume -> increased blood viscosity -> increased cardiac workload
- poor O2 delivery to organs -> ischemia and thrombosis
types of polycythemia vera
- primary: EPO independent
- secondary: EPO driven
primary polycythemia vera
- constant RBC production independent of EPO
- JAK2 is activated by EPO in normal pts
causes of secondary polycythemia vera
- hypoxia
- EPO secreting tumors
- adrenals: adenoma, cushing’s
- renal causes
- testosterone
classic presentation of polycythemia vera
- severe burning in hands/ feet
- red or blue skin discoloration
- aka erythromelalgia
- aquagenic pruritis
sx of altered BF and cell proliferation in polycythemia vera
- HA
- sweating
- tinnitus
- blurred vision/ blind spots
- dizziness, vertigo
- reddish or purplish skin
- splenomegaly, hepatomegaly
sx of increased platelet levels in polycythemia vera
- thrombotic events
- bleeding
- ACS
- CHF
- inflammation
treatment of polycythemia vera
- phlebotomy
- low dose ASA
- if high risk thrombosis/ poor compliance hydroxyurea to suppress bone marrow
