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S3.M3.Path 1
RBCs and Coagulation disorders
43
Pathology
Professional
07/10/2010

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Cards

Term
perforate basophilia--> what is it, what causes it?
Definition
bunches of ribosomes in RBCs due to denatured RNA caused by LEAD POISONING
Term
SCHISTOCYTES-->what are they, what causes them?
Definition
fragmented RBCs, cause by hemolytic anemia (microangiopathic DIC, TTP, HUS)
Term
what is a NORMOBLAST in a PBS?
Definition
nucleated RBC --> looks like a lymphocyte, caused by massive hemorrhage or hemolysis
Term
what are HEINZ BODIES? causes?
Definition
they are seen using Supra-vital stains (crystal violet) -->Particles of denatured hemoglobin on inner face of RBC membrane--> seen in G6PD
Term
what are SIDEROCYTES? causes?
Definition
iron accumulations in RBC not Hb bound, caused by iron overload (mitochondria) --> prussian blue stain
Term
polychromasia
Definition
persistence of RNA and may be the sign of incomplete cell maturation or more immature red cells being released into the circulation (reticulocytes)
Term
what is PNH? causes?
Definition
paroxysmal nocturnal hemaglobinuria-->complement mediated RBC damage, (PIGA gene deficiency)
Term
what are two causes of raised RETICULOCYTE levels?
Definition
blood loss, hemolysis
Term
pernicious anemia--> describe
Definition
autoimmunity causes atrophic gastritis which affects PARIETAL cells (intrinsic factor)--> no B12 absorption
Term
seen in 1)low folate or 2) low B12 or both:
-raised homocysteine
-raised methylmelonic acid
-parasthesis and tingling in lower limbs
Definition
-raised homocysteine --> BOTH
-raised methylmelonic acid --> VIT B12
-parasthesis and tingling in lower limbs--> VIT B12
Term
3 cells seen in MACROCYTIC ANEMIAS?
Definition
megaloblastic anemias--> BM
macrocytes and hypersegmented neutrophils --> PBS
Term
signs of PERNICIOUS ANEMIA?
Definition
same as megaloblastic anemias (megaloblasts, macrocytes, hyperseg neutrophils) + possible pancytopenia + ACHLOHYDRIA (pH>4)
Term
where are the following three absorbed?
-iron
-folic acid
-vit B12
Definition
-iron-->duodenum
-folic acid-->jejunum
-vit B12-->illeum
Term
clinical findings of IRON DEFICIENCY?
Definition
HYPOchromic microcytic anemia, atophic glossitis, spoon shaped nailbeds, PICA
Term
what is PLUMMER WINSON SYNDROME?
Definition
Fe deficient anemia, esophageal webs-->dysphagia
Term
what is HEPCIDIN?
Definition
IRON REGULATOR made in liver --> important in CHRONIC ILLNESSES because it works to decrease serum iron--> less iron for pathogens-->iron deficient anemia
Term
clinical findings in LEAD POISONING?
Definition
abd. colic, encephalopathy, Pb deposits in epiphysis of bone--> screened by BLOOD LEAD levels
Term
Thalassemia major clinical findings
Definition
hypochromic microcytic anemia, HbF*** some HbA2 (minor), splenomegaly, extra medullary hematopoiesis, excess Fe absorption
Term
serum Fe, TIBC and serum Ferritin levels in:
-iron deficient anemia
-chronic illness
-sideroblastic anemia
Definition
-iron deficient anemia-->low Fe, ferritin, high TIBC
-chronic illness-->low Fe, TIBC, high ferritin
-sideroblastic anemia -->low TIBC, high Fe, ferritin
Term
serum Fe, TIBC and serum Ferritin levels in:
-iron deficient anemia
-chronic illness
-sideroblastic anemia
Definition
-iron deficient anemia-->low Fe, ferritin, high TIBC
-chronic illness-->low Fe, TIBC, high ferritin
-sideroblastic anemia -->low TIBC, high Fe, ferritin
Term
thalassemia causes (alpha vs. beta)
Definition
alpha thal=deletions
beta thal=point mut.-->splicing defects
Term
what is HAPTOGLOBIN? when are HAPTOGLOBIN levels reduced?
Definition
plasma protein in charge of clearance of free Hb--> reduced levels in INTRAVASCULAR HEMOLYTIC ANEMIA
Term
how is SPHEROCYTOSIS treated?
Definition
splenectomy
Term
what tests and PBS findings indicate Her Spherocytosis?
Definition
normal HCV, raised reticulocytes, + osmotic fragility test
Term
what is APLASTIC CRISIS?
Definition
complication of H spherocytosis caused by PARVOVIRUS infection
Term
what is CHOLETHIASIS? when is this seen in blood disorders?
Definition
gall stones-->hemolytic anemias when unconjugated bilirubin binds with calcium in gall bladder
Term
what is the major complication involved with SCA?
Definition
vaso-occlusion crisis--> dactylitis, infarction of any organ
Term
what happens with SPLEEN in older SCA patients?
Definition
atrophies and dies--> autosplenectomy due to infarction that kills organ
Term
how is SCA screened for and confirmed?
Definition
screened with SICKLING TEST, confirmed with ELECTROPHORESIS for high HbS levels
Term
mechanism of PNH
Definition
PIGA mutations--> no GPI anchor protein--> nothing to hold CD 55 & 59* in place--> complement binding and destruction
Term
how is PNH confirmed?
Definition
HAM'S test
Term
what is seen in the PBS of G6PD?
Definition
heinz bodies and bite cells--> G6PD can be induced by ANTI-MALARIAL DRUGS
Term
2 drugs related to immune hemolytic anemia?
Definition
penicillin, methyldopa--> both cause type II HS
Term
most classic example of hemolytic anemia-
Definition
SLE--> warm antibody (most active in warm temps) IgG**
Term
what type of hemolysis is caused by MYCOPLASMA?
Definition
Immune hemolytic anemia--> cold agglutination type --> IgM--> autoagglutinate when in cold (near peripheries) --> cause infarction
Term
causes of Aplastic anemias?
Definition
-CHEMICAL (benzene)
-INFECTION (parvovirus B19)
-HEREDITARY (fanconi's anemia)
Term
findings in the PBS of aplastic anemia patient
Definition
pancytopenia including low reticulocyte
Term
findings in the PBS of aplastic anemia patient
Definition
pancytopenia including low reticulocyte
Term
what is MYELOPHTHISIC ANEMIA? findings in PBS?
Definition
lesion in BM like metastatic cancer or myelofibrosis -->dacrocytes, immature erythroid and myeloid precursors in PBS
Term
what disease should you think of when you hear FIBRIN DEGRADATION PRODUCTS? (D dimer assay)
Definition
DIC
Term
if bleeding times and PT/PTT are normal what should you think?
Definition
vascular disorders
Term
ITP mechanism
Definition
ITP= immune thrombocytopenic purpura--> IgG binds glycoprotein (GP) IIb-IIIa or Ib-IX platelets and is destroyed in spleen
Term
what disease would a normal PT but high PTT Indicate?
Definition
hemophelia --> factor VIII
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