Blood Cell Disorders--Vo Flashcards
a reduction in one+ of the major RBC measures
Anemia (RBC count (rarely used), Hct, Hgb) (most common hematologic disorder
“normal” Hct/Hgb levels influenced by (5)
PREGNANCY (37/12), sex, ^ altitude, hydration status, and age
damage to cells seen on blood smear (3), all schizocytes
- bite cells
- helmet cells
- triangle cells
RBCs w/ nuclear remnants, usually cleared out by spleen, but sign of cells leaving bone marrow while incompletely mature–possibly pointing to ________ ________
Howel Jolly bodies,
spleen absence
Acnthocytes:
Spur cells indicate:
Burr cells indicate:
- liver disease
2. renal failure
reticulocytes mature to RBC over ____ days
four
normal reticulocyte release:
acute anemia reticulocyte released:
- w/ 1 day left to mature
2. X 2-3 production released early
*reticulocyte count expressed in
% of total RBCs – 1-2% of Hct normally
Corrected:
% retics measured X Pt Hct/ normal Hct
retic index tells you
how the bone marrow is responding to the anemia.
absolute (corrected) reticulocytes count : # of days til mature
Reticulocyte index:
1 =
>2 =
<2 =
- normal
- adequate marrow response
- hypoproliferative marrow
Iron %’s (3)
- 65% in hemoglobin in RBCs
- 4% in myoglobin
- 30% stored as ferritin or hemosiderin in spleen, marrow, and liver
Transferrin =
iron + apotransferrrin
measurement of total available spaces for Fe to bind (total empty spaces remaining in the apotransferrin molecule)
Total Iron Binding Capacity
The Iron Panel (3)
- Serum Iron
- Total Iron Binding Capacity
- Ferritin (total stored iron)
test to detect antibodies that are already bound to the surface of red blood cells
Direct Comb’s test
test that looks for unbound circulating antibodies in serum against the RBCs
Indirect Comb’s test
Test that separates hemoglobin species according to aa composition (globin subunits)
Electrophoresis
Microcytic anemia MCV
<80
Cx’s of Microcytic anemias (5)
- *Fe deficiency anemia
- Anemia of Chronic Dz’s / Inflammatory Dz’s
- Thalassemia
- Sideroblastic anemia
- Lead Poisoning
Loss of RBC membrane size and/or structural integrity may lead to
Spherocytes
causes of spherocytes (2)
- hereditary spherocytosis
2. autoimmune hemolytic anemia
irregular adherence of RBCs cx from elevated plasma Globulins called ________ _________ seen in:
Rouleaux Formations,
Multiple myeloma
Lot’s of blue means…
lots of new
Normocytic anemias MCV
80-100 – Many cx’s slide 46
Macrocytic anemia MCV:
causes (3)
> 100,
- Vit B12 deficiency
- Folic acid deficiency
- Myelodysplastic disorders
Thalassemia
a blood disorder passed down through families (inherited) in which the body makes an abnormal form of hemoglobin, the protein in red blood cells that carries oxygen. The disorder results in excessive destruction of red blood cells, which leads to anemia
most common cause of anemia worldwide
iron deficiency anemia (a complication not a dx)
Cx’s of iron deficiency anemia (5) (slide 49)
- Chronic blood loss
- malnutrition
- malabsorption
- increased demand
- Fe+ sequestration (by liver or spleen)
Early Iron deficiency anemia (5)
- only iron stores low
- low ferritin
- serum Fe normal
- TIBC rises
- normal MCV
Stages of Iron deficiency (3)
- Early
- Iron deficient erythropoiesis
- iron deficiency anemia
esophageal webs aka
Plummer-Vinson Syndrome
Tx for Iron deficiency anemia (3)
- tx underlying cause
- Iron replacement (Fe Sulfate – DI PPI’s–need acidic environment)
- Repeat CBC in 2-3 months
Other iron formulations (3)
- Gluconate
- Fumarate (time-release)
- Parental (veno-toxic)
iron absorbed in
proximal small intestine–thus in pt’s w/ gastric bypass give IV or higher doses of Iron
Anemia of Chronic/ Inflammatory Dz def
Anemia occuring as a complication of:
- chronic infxn–bac endocarditis, lung abscess
- inflammation–arthritis
- Cancer–Hodgkin dz, carcinomas
- Liver Dz–cirrhosis
inherited disorders involving gene deletions or point mutations resulting in a defect in the structure, function, or production of Hgb
Hemoglobinopathies–leads to anemia or even hemolysis
Hemoglobinopatheis (6)
- Thalassemia (alpha & beta) MOST COMMON
- Sickle cell
- Hgb C dz
- Hgb D dz
- Hgb E dz
- Hereditary spherocytosis
decreased production of 1,2,3,4 alpha-chain genes
Alpha Thalassemia (southeast asia or china)
(4) types of alpha thalassemia
- silent carrier or alpha thalassemia-2 (aa,a-)
- Alph thalassemia-1 (a-,a-) (aa,–)
- Hemoglobin H Dz (a-,–)
- Hydrops Fetalis (hemoglobin Barts) (–,–) stillborn
Alpha Thalassemia Traits (5)
- mild anemia
- MCV LOW
- HYPOCHROMIC MICROCYTES
- TARGET CELLS
- you will see a PROPORTIONALdecrease in all type of hemoglobin
if spleen is removed vaccinate for
encapsulated organisms
Tx for Alpha Thalassemia
No tx necessary–genetic counseling
Alpha thalassemia has 4 genetic possibilities BUT 2 possible clinical presentations.
(xx,x-) asymptomatic
(–,–) stillborn
Beta thalassemia
gene deletions and point mutations result in reduced or absent beta synthesis
beta thalassemia seen in
- persons of greek and mediterranean decent
- chinese, black, asians less common
hemoglobin ratios in beta thalassemia
increase in the % of Hgb A2 and F compared to hemoglobin A
(3) types of Beta Thalassemia
- trait/ minor (B+,B)(B0,B) MCV 55-75
- Intermedia (B+,B+)(B0,B+) 2 mutations but milder
- Major (B0,B+)(B0,B0) (Cooley’s Anemia) severe anemia
Blood smear is “crazy weird”
Beta Thalassemia
basophile stippling =
lead poisoning
those anemieas which result from an ^ in the rate of red cell destruction
hemolytic anemias
normal adult marrow is able to produce RBCs at _________ X the normal rate
6-8X
(2) types of hemolytic anemias
- intrinsic cx
2. extrinsic cx
G6PD deficiency most common in
Mediterranean and Chinese populations (may see neonatal jaundice)
Hct decrease faster than 3%/week indicates (2)
- blood loss
2. hemolysis– <3%/week = not hemolysis
Stand-out Sickle cell Dz symptoms (6)
- jaundice
- pigment gallstones
- splenomegaly (early on)
- ulcers over lower leg
- non healing ulcers on lower legs
- RETINOPATHY
dactylitis seen in
Sickle cell Dz
Splenic infarction causes infxn by
encapsulated organisms (N. meningitidis, Strep, Hemophilus, Klebsiella)
Pt w/ salmonella osteomyelitis think
Sickle Cell!
“old school” method of Dx sickle cell
sickle-Dex test
- Tx mainstay of sickle cell
HYDROXYUREA (suppress WBCs, cytotoxic) +
O2, hydration, pain management
Parvovirus B19 w/ sickle cell trait think
Aplastic crisis (transient arrest in erythropoiesis w/ marked decrease in reticulocytes) r/o w/ retics
Most common hemolytic anemia in North Europeans
Hereditary Spherocytosis (auto dom)
hereditary spherocytosis cx from
defect in the membrane protein responsible for the cytoskeletal structure and stability. Decreased SA:V
Spherocytes are
unable to pass through spleen and are destroyed
Hereditary spherocytosis clinical features (4)
- jaundice
- splenomegaly
- gallstones (pigmented from ^ bilirubin)
- Scleral icterus
the only anemia where MCHC is increased
Hereditary Spherocytosis
Tx for Hereditary spherocytosis (3)
- folate
- splenectomy
- possible cholecystectomy
G6PD deficiency seen in
males of African or Mediterranean descent (X-linked)
The G6PD enzyme is critical to
the metabolism w/in cells and protects the cells from oxidative stresses–>hemolysis of RBCs
G6PD deficiency blood smear
Heinz bodies (oxydized Hgb)
Most common form of acquired hemolytic anemia (besides malaria)
Autoimmune hemolytic anemia AIHA
Warm AIHA mediated by
IgG –37C–RBC destruction in spleen (EXTRAVASCULAR)–ACUTE ANEMIA, SPLENOMEGALY (distinguishing)
Cold AIHA mediated by
IgM–<37C–RBC destruction in blood vessels (INTRAVASCULAR)–DECREASED HAPTOGLOBIN (distinguishing)
Warm AIHA Tx (5)
- Glucocorticosteroids (rapid taper)
- Chemo
- IV Ig
- Splenectomy
- support RBC production (folate)
for AIHA Comb’s test will be positive in
Cold and Warm AIHA–history and physical most important dx factor
Megaloblastic anemias (2) Roughly the same presentation except:
- Folate deficiency (NO NEURO SYMP’s)
- Vit B12 deficiency (NEURO SYMPTOMS, hyperseg neuts)
(Defective DNA synth –>disordered RBC maturation–>cytoplasmic RNA acummulation–>larger RBC’s
*Megaloblastic WBCs on smear
Neutrophils w/ 5+ lobes
Neuro effects of vit B12 deficiency
Myelin breakdown –> neurological symptoms
Vit B12 absorbed in
terminal ileum
Cx of Vit B12 deficiency (rare)
- gastrectomy
- nutritional (vegan, alcoholism)
- Terminal ileal resection
- CROHN’S DZ
- Competing organisms (worm, bac)
- Medications (PPI’s, METFORMIN)
autoimmune disorder disallowing against INTRINSIC FACTOR–> Can’t absorb B12
Pernicious Anemia
HIGH METHYLMALONIC ACID w/ megablastic anemia
B12 deficiency
Schilling test for
B12 deficiency (radioactive labeled B12, ABX, determines if bac causing low B12)
Dx of pernicious anemia (3)
- EGD (visualize gastritis changes)
- Bx
- measure anti-parietal cell antibodis
B12 storage:
Folate storage:
- 2-3 years
- 3 months
syndrome of chronic primary hematopoietic failure and attendant pancytopenia (low RBCs, WBCs, and platelets)
Aplastic Anemia (1000 cases in US/yr– 15-30yo’s and >60 yo’s)
etiologies of aplastic anemia (5)
- inherited
- Idopathic
- Medications (Chemo)
- Viral (B12, Hep, CMV, HIV)
- Radiation exposure
bone marrow failure consequences (aplastic anemia)
- anemia
- neutorpenia (recurrent infxn)
- thrombocytopenia
if Cafe au lait spots and short stature w/ aplastic anemia think
Fanconi’s anemia (genetic)
Only Tx for Aplastic Anemia
bone marrow transplant
A myeloproliferative disorder of the hematopoietic progenitor cells in which there is overproduction of phenotypically normal red cells, granulocytes, and pltatelets
Polycythemia Vera (mostly RBC line effected)
Dx of polycythemia vera (3)
- PRURITUS (aguagenic)
- hepatosplenomegaly
- Neuro (headache, dizziness, tinnitus, fatigue)
DDx polycythemia vera (2)
- CML (PHILADELPHIA CHROMOSOME)
2. secondary polycythemia (Hyposemia)
Polycythemia vera Labs (4)
- Hct >50%
- platelets >400K
- WBC >12K
- high B12
Tx for polycythemia vera ()
- prevent thrombosis from hyperviscosity
- Myelosuppretion (damage bone marrow to decrease overproduction)
- ANAGRELIDE (decrease platelet count)
