Polycythemia Vera and Hemochromatosis Lecture Powerpoint Flashcards
Craig is bae
Hct is also known as
Pack cell volume (PCV)
Polycythemia vera defintion
A myeloproliferative blood cancer characterized by excessive proliferation of all blood elements in bonemarrow producting erythrocytosis, leukocytosis, and thrombocytosis
Polycythemia vera sequale (4)
- splenomegaly
- claudication
- hyperuricemia (kidney stone or gout development)
- Myelofibrosis (fibroses of bone marrow leading to aplastic anemia)
What causes polycythemia vera?
JAK 2 Mutation of hematoopoietic stem cells that causes proliferation independent of erythropoietin levels
Polycythemia vera is most often seen in this age group
50-70
Erythropoietin levels in polycythemia vera
Low
Signs and symptoms of polycythemia vera (7)
- headaches
- vertigo
- blurred vision
- fullness in head and left upper quadrant
- red discoloration esp. of face
- oozing from gums
- epistaxis
Classic sign of polycythemia vera
Pruritis often unbearable after bathing due to warm water facilitating excessive histamine release in overabundance of mast cells
Ecchymoses definition
Bruising
Gout definition and where is it commonly seen
Build up of uric acid crystals causing inflammation, frequently seen in joints such as 1st metatarsal phalangeal joint
Prognosis of polycythemia vera with and without treatment
Serious disease with survival of only 6-18 months with no treatment, can add 12 years of life with treatment
Treatment of polycythemia vera (5)
- Phlebotomy to decrease hematocrit btwn 40-50
- splenectomy
- prophylactic treatment against thrombosis with aspirin
- histamine blockers for pruritus
- hydroxyurea as myelosuppression
Hemochromatosis definition
Bronze diabetes, genetic disorder of iron metabolism causing body to store excess iron resulting in overload
Hemochromatosis onset
Doesn’t appear until after years of build up in middle ages
Signs and symptoms of hemochromatosis (4)
- skin pigmentation
- arthalgia
- diabetes type I
- impotence
