Red Cell Disorders 4 Flashcards
once iron is absorbed in the _______, it can follow two pathways:
duodenum
- transport to the blood
- storage as mucosal iron
hepcidin is synthesized in response to high levels of ______ iron stores and inhibits _______
liver iron stores;
iron uptake from duodenal mucosal cells into the bloodstream
normally _____% of the transferrin in saturated with iron
33%
patient is easily tired and has a constant desire to eat ice. What is the most likely diagnosis
iron deficiency anemia;
ice craving is specific for iron deficiency anemia
Plummer Vinson Sydrome has a triad of: _____, _____, and iron deficiency anemia
- dysphagia
- esophageal webs
low/high RDW is seen with iron deficiency anemia
high
low/high reticulocyte count is seen with iron deficiency anemia
low
what type of chronic diseases is associated with anemia of chronic disease
- chronic bacterial infections: lung abscesses, osteomyelitis and endocarditis
- chronic immune disorders: RA
- malignant tumors: lymphomas, lung or breast cancer
what is the pathogenesis of the anemia of chronic disease?
- impairment of iron utilization and reduction in RBC production:
- inflation will inc. production of hepcidin and thus causes dec. production of erythropoietin
discuss the levels of ferritin, iron, TIBC and transferrin saturation in anemia of chronic disease
- ferritin: high (functional IDA; due iron not being utilized and decreased RBC production)
- iron: low (sequestration of iron from the circulation)
- TIBC (low)
- transferrin saturation (normal)
transferrin saturation is a ratio of ____ to ______
serum iron: TIBC
list the clinical features of aplastic anemia
- symptoms of anemia
- recurrent infections (do to neutropenia)
- mucosa hemorrhage or menorrhagia (due to thrombocytopenia)
- NO ORGANOMEGALY
“dry tap” of bone marrow aspiration is seen in _____
aplastic anemia because the bone marrow is filled with fat
what is myelophthisic anemia?
bone marrow replacement by abnormal infiltrates that disturb the normal architecture (often with fibrosis)
causes of bone marrow failure:
- aplastic anemia
- myelophthisic anemia
- diffuse liver disease
- chronic renal failure
leucoerythoblastic picture is seen with ________ anemia
myelophthisic anemia
how would a patient with polycythemia present?
- hyper-viscosity symptoms: headache, dizziness, visual disturbances
- pruritus
- early satiety due to splenomegaly
- thrombosis/bleeding
relative polycythemia is secondary to _____
dehydration;
relative polycythemia is due to reduced plasma volume
_______ absolute polycythemia is associated with LOW EPO levels and ______
primary (aka polycythemia vera); primary absolue polycythemia id due to a chronic myeloproliferative neoplasms
SPLENOMEGALY
what are some secondary causes of polycythemia with appropriately high EPO levels
- lung dise ase
- cyanotic heart disease
- living at high altitude
what tumors are associated with EPO secreting tumors
- renal
- cerebellar
- hepatic
______ mutation is found in 98% of people with polycythemia vera
JAK2 mutation
causes of bleeding disorders (3)
- increased small blood vessel fragility
- platelet dysfunction or deficiency
- coagulation abnormality
thrombin time tests _____
fibrinogen
what are 4 categories for the causes of thrombocytopenia
- decreased platelet production
- decreased platelet survival
- splenic sequestration of platelets
- dilutional thrombocytopenia
immune thrombocytopenic purpura is due to antibodies directed against ________
platelet membrane glycoproteins IIb/IIIA or Ib/IX complexes
describe the role of the spleen in immune thrombocytopenic purpura
- site of anti platelet antibody production
- site of destruction of IgG coated platelets
________ bone marrow is seen in immune thrombocytopenic purpura
megakaryocytic hyperplasia due to the excessive peripheral destruction of platelets
________ can induce a remission fo chronic ITP
splenectomy
what would you see on bone marrow biopsy of someone with immune thrombocytopenic purpura?
- inc. megakaryocytes
describe the mechanism of thrombocytopenia due to non immune thrombocytopenia: TTP/HUS
- there is widespread formation of platelet rich thrombi in small blood vessels → platelet consumption → thrombocytopenia
give examples fo hereditary clotting factors
- Factor VIII
- Von Willebrand Factor
- Factor IX
what are some acquired clotting disorders
- vitamin K deficiency
- severe liver disease
- DIC
hemophilia A is due an abnormality in )____
factor VIII
Factor VIII is a cofactor for Factor IX to activate factor X in the intrinsic pathway
8+9 = 10
what are the functions of VWF?
- promotes adhesion of platelets to the subendothelial layer after damage
- prolongs the half life of Factor VIII
type _____ of von willebrand disease is associated with reduced quantity of VWF
types 1 and 3
Type _____ of von willebrand disease is associated with qualitative/ functional abnormality of VWF
type 2
VWF prolongs the half life of _____
Factor VIII
the most severe type of VWD (von willebrand disease) is type _____
type 3 because it has a severe deficiency or lack of VWF → presents very similar to Hemophilia A
what the lab findings for someone with VWD:
bleeding time
PT:
PTT
- bleeding time: normal
- PT: normal
- PTT: normal or ↑
describe the clinical presentation of someone with hemophilia A
- massive bleeding after trauma or surgery
- “spontaneous” hemorrhages usually in joints or muscles
- NO PETECHIAL HEMORRHAGES because no problem with platelets
