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99 Cards in this Set

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Abnormal / unequal size
Nonuniform shapes in TTP/HUS, microvascular damage, DIC
Results from decreased RBC membrane

Seen in hereditary spherocytosis and AIHA
Target cells
Result from increased RBC membrane

Seen in hemoglobinopathies (HbC), Asplenia, Liver Dz, and thalassemias
Have irregular specules on their surface

Seen in abetalipoproteinemia
Mechanical destruction of RBC, resulting in RBC fragments (helmet cells)

Seen is micro- and macro-angiopathic hemolytic anemia or traumatic hemolysis, and DIC
Teardrop cells
Thalassemia and Myeloid metaplasia with myelofibrosis
Stack of coins - RBCs lining up in a row

Seen in MM
Basophilic stippling
Results from cytoplasmic remnants of RNA

Indicative of reticulocytosis or lead poisoning
Howell-Jolly bodies
Remnants of nuclear chromatin

Indicative of severe anemias or asplenic pts
Pappenheimer bodies
Composed of iron

Found in pts following splenectomies
Ring sideroblasts
Iron trapped abnormally in mitochondria, forming a ring around the nucleus

Indicative of sideroblastic anemia
Heinz bodies
Denatured Hb

Indicative of G6PD deficiency
What is the DF of anemia
Reduction below NMl limits of total ciculating red cell mass:
Males: Hb < 13.5, Hct<41
Females Hb < 12 Hct <36
Signs of anemia
Pallor of skin
Koilonychia (spoon nails)
Epithelial atrophy seen in PLummer-Vinson syndrome
Megaloblastic anemia (also hyperpigmented PMNs), marrow failure
Burr cell
Reticulocytes count
% of RBCs in the peripheral blood
Name the micorcytic anemias and associated pathogenesis
These are hypochromic and MCV <80

Pathogenesis = defect in synthesis of Hb (heme + globulin chains
Heme = iron + protoporphyrin

Iron deficinecy - heme syn defect
Thalassemia (target cell) - defect in globulin chain
Sideroblastic anemia - defect in heme syn
Anemia of chronic Dz - defect in heme syn
Causes of iron deficiency anemia
Diet deficiency
Increased demand - children, and pregnant
Malabsorption or after gasterectomy
Chronic blood loss - menstural or CA

MC caused by blood loss
Lab values' sequnce of events in iron deficiency anemia
1. Decr storage iron - Decr ferritin
2. Decr circulating iron - Decr serum iron & incr total iron binding capacity (TIBC) & decr % saturation
3. Decr MCV, Decr MCHC, High RDW
3 main causes of ACD?

Pathogenesis of anemia of chronic Dz?
1. Chronic Inflammation
2. ETOH (MC type in alchol)
3. Malignancy (MC type in malig)
4. Hospitalized pts (MC type in hosp pts)

Inflammation - liver release of hepcidin (acute pahse reactant) - enters macro and prevents the release of iron TO transferrin - incr iron stores in bone marrow macrophage
Lab value of anemia of chronic Dz
Incr serum ferritin

Decr Serum iron, TIBC, and % saturation
Lab values of sideroblastic anemia
Incr: Serum iron, ferritin, and % iron sat
Decr TIBC and MCV

Ringed sideroblasts are present in bone marrow aspirate
What are the macrocytic anemias
MCV >100
1. Megaloblastic (B12 and folate deficiency) - hypersegmented PMNs
2. Alcholic Liver Dz
3. Drugs that black DNA synthesis (AZT, sulfa drugs)
4. Marked reticulocytisis (bigger than mature RBCs)
After determining pt has a normocytic anemia what is the next thing to determine?
Reticulocyte count - either Low or High, categorizes the normocytic anemias
What are the Normocytic Low corrected reticulocyte count anemias (<3%)?
Aplastic anemia
Marrow failure
Early Anemia of Chronic Dz
Renal failure
What are the Nomocytic High Correcetd reticulocyte count anemias (>3%) Intrinsic RBC defect
Sickle cell
G6PD deficiency
Hereditary sphirocytosis
Pyruvate kinase deficiency
What does decr serum haptoglobin and incr serum LDH indicate?
F/U Test?
RBC hemolysis
F/u with direct Coombs' test to distinguish b/w immune vs. non-immune mediated RBC hemolysis
What is the Df of aplastic anemia?
Pancytopenia - severe anemia, neutropenia, and throbocytopenia due to failure or destruction of the multipotent myloid stem cell.
Causes of aplastic anemia?
Radiation, benzene, chlorampenicol, alkylating agents, parvovirus B19, EBV, HIV, Fanconi's anemia
Sx of aplastic anemia
pallor, petechia, purpura, infection
Pathologic features of aplastic anemia
Dx: Bone marrow biopsy - hypocellular bone marrow with fatty infiltration, pancytopenia with normal cell morphology
Tx for aplastic anemia?
Allogenic bone marrow transplant
RBC and platlet transfusion
What type of anemia does blood loss result in?
Normocytic anemia
NOTE: chronic blood loss from menses or GI bleed may cause iron deficiency anemia (microcytic)
What is the mutation responsible for sickle cell
AR: glutamic acid (-charge)to Valine (neutral) to at position 6 on the beta chain
Which pts suffer Sx of sickle cell and which have milder Dz
SS (Hbs) homozygotes

NOT AS heterozygotes,
HbC, and HbSC (one of each mutant gene) have milder Dz than HbS
NOTE: HbC is the result of a different Beta chain mutation
what preceipates the Sx of sickle cell
Anything that cause incr in DEOXYHb:
1. Dehydration
2. Acidosis decr o2 affinity for RBC
3. Infection
4. Hypoxemia - Decr arterial O2 decreases O2 SAT of Hb
Clinical findings of sickle cell
Most often in HbSS (homozygotes)
1. Aplastic crisis (B19 infection)
2. Autosplenectomy - will see Howell-Jolly bodies, and subsequent incr infection with encapsulated organism (s. pneumo sepsis)
3. Vaso-occlusive crisis (painful)
4. Salmonella paratyphi osteomylitis
5. Priapism
6. Acute chest syndrome (Vaso-occlusion of pulm capillaries, chest pain / lung infiltrates / hypoxemia, MCC of death in adults)
7. Aseptic necrosis of the femoral head
Molecular bio technquie for deciphering b/w HbA (alpha2, beta2) and HbS
Electrophoresis - HbS migrates further than HbA
Mutation reponsible for HbC
Lysine (basic) replaces glutamic acid at the beta 6 position
Sx of HbC Dz
Mild normochromic, normocytic anemia
Target cells
Rod shaped crystals in RBCs (characteristic)
In what parts of the world is alpha and beta thal most prevalent in
alpha - Asia and Africa

Beta - Meditarranean
What are the different varaints of alpha Thal
Normal: 4 alpha genes
Silent carrier: One deletion, 3 alpha genes
Alpha Thal trait: Two deletions, 2 alpha genes, cis genotype (--/aa) in Asians and tran genotype (-a/-a) in African-Americans - present with mild anemia
HbH Dz: 3 deletions, 1 alpha chain, tetramer of Beta chains form, Heinz bodies and severe hemolytic anemia
Bart Hb / Hydrops: 4 deletions, gamma4 tetramers, lethal in utero
What is the clinical significance of the formation of Beta tetramers in HbH Dz
Decreased O2 binding - precipiate - lysis
What is the difference in timeline b/w when alpha Thal and Beta Thal present?
Alpha Thal presents shortly after birth since alpha chains are expressed prenatally

Beta thal (Major) 6 mo after birth when HbF level decrease
What characterizes Beta Thal minor?
One mutated allel (heterozygote)
Increased HbA2 (alpha2, delta2)
Increased HbF (alpha2, gamma2)
Asx or slight anemia
What characterizes Beta Thal major?
Both beta chains are mutated (homozygote) and therefore absent, therefore alpha chain ppt and lyse resulting in severe hemolytic anemia (microcytic, hypochromic) and jaundice
What are the conseqeunces of Beta Thal major?
Life long transfusions, Cardiac failure secondary to hemochromatosis,
Erythroid hyperplasia in the bone marrow causes CREWCUT skull x-ray and increased size of Maxilla
What are the 2 general categories of hemolysis?

2 categories of pathogenesis?
Intravascular hemolysis - characterized by decr haptoglobin and hemoglobinuria

Extravascular hemolysis (i.e RBC destruction in spleen or liver Kupffer cells) characterized by unconjugated hyperbilirubinemia

Extrinsic - immune destruction or calcific aortic valve

Intrinsic - membrane defects, abn Hb, enzyme deficiency
What are the 4 major types of Hemolytic anemia
Hereditary Sperocytosis
Paroxysma nocturnal hemoglobinuria
Microangiopathic hemolytic anemia

Sickle cell, Thalasemmia, G6PD, HbC
What are the 3 main types and characterisitcs of the AIHAs
Warm aggultinin - IgG Ab, are active at 37C; ONLY IgG-mediated is associated with spherocytosis, also associated with SLE, CLL, small lymphoctic lymphoma, drugs (PCN and alpha-methyldopa)
Dx: Positive Direct Coombs' test aka DAT (antiIg Ab added to pts RBCs and will agglutinate if RBCs are coated with Ig)

Cold Agglutinin - IgM Ab, anemia triggered by cold; associated with M. pneumonoia and IM

Erythroblastosis fetalis: Mother's Ab attack fetal RBC due to Rh incompatability

All of these are characterized by extravascular hemolysis - RBC destruction in Liver Kuppfer cells and spleen

AIHA are Coombs positive (Direct and Indirect)
Indirect: Normal RBCs added to pts serum agglutinate if serum has anti-RBC surface Ig
Hereditary spherocytosis
Intrinsic, extravascular hemolysis

AD disorder, defect involving spectrin or ankyrin in RBC membrane, causes a decrease in the RBC surface membrane

RBCs are small, round with no central pallor - less membrane - incr MCHC and incr RDW

Dx: Coombs' test negative, Increased osmotic fragility test used to confirm (high osmotic fragility--when placed into water, they are more likely to burst than normal red blood cells b/c incr water permeability due to dysfxnal Na/K ATPase)

NOTE: Both Warm AIHA and Hereditary spherocytosis have spherocytes in peripheral blood - Coombs' test and osmotic fragility test to distinguish
Paroxysmal nocturnal hemoglobinuria
Intravascular hemolysis, Intrinsic defect

Decreased Decay accelerating factor, which nmlly inhibits activation of complement by breaking down C3 convertase
Deficiency = incr lysis of RBCs

Acidosis that occurs at night causes activation of compliment

Lab: Incr urine Hb, pancytopenia in peripheral blood
Pathogenesis of G6PD deficiency
X-linked deficiency leads to decreased NADPH production, unable to keep GSH reduced, cell damage from peroxide b/c absence of GSH protection

Intrinsic defect, intravascular and extravascular hemolysis
What ppt hemolysis in pts with G6PD

Things which incr oxidative stress:
1. Infection
2. Drugs (primaquine, sulfonamides, anti-TB, dapsone)
3. Fava bean

Sudden onset back pain + hemaglobinuria 2-3 days after an oxidant stress
Microscopic evidence of G6PD induced hemolysis
Heinz bodies seen with methylene blue stain

Peroxide oxidizes Hb - Heinz bodies - Heinz bodies damage RBC membrane causing intravascular hemolysis - Heinz bodies removed from RBC membrane by splenic macrophage (extravascular hemolysis) producing BITE CELLS (macrophage removal of membrane)
Causes of DIC
Retained fetus
amniotic embolism
abruotio placenta
Gram negative sepsis (E. coli, NG), Ricketssia
acute pancreatitis
Nephrotic syndrome
DIC lab findings
Incr: PT, PTT, D-dimers
Decr: Platlets
Causes of B12 deficiency
1. Diet - Only in vegans (No meat, milk, or egg

2. Decr absorption caused by:
a. Decr IF with gastectomy (parietal cells syn IF) or pernicious anemia
b. Pancreatic insuff - proteases breakdown B12-R complex
c. Malab due to bacteria or chrons Dz (terminal illium)
S/S of B12 deficiency
1. Megaloblastic anemia - weakness, etc
2. Sore beefy tongue due to generalized epithelial atrophy
3. Subacute combined spinal cord degen: posterior columns (vib / prioprioception) + Lateral (dorsal spinocerebellar tracts - arm and leg dystaxia and CST - spastic paralysis)
4. Pernicious anemia characterized by: Achlorhydia and destruction of parietal cells causing atrophic gastritis with an incr risk of adenocarcinoma
5. Hypersegmented PMNs
Lab results of B12 deficiency
Increased methylmalonic acid in urine

Schilling Test: Looking for urinary excretion of B12
1. B12 alone - low urine
2. B12 w/IF - Nml urine B12 = pernicious anemia
3. If #2 is low give abx - nml urine B12 = bacteria over growth
4. If #3 still low - chrons or other problem with terminal ilium
4 main causes of Folate deficiency anemia
1. Decreased Intake - chronic alcholics and elderly on tea and toast diet
2. Decreaed absorption - intestinal malab of proximal small intestine uptake (celiac dz)
3. Increased requirements - Prenancy and infants
4. Drugs
5-FU: Inhibits thymidylate synthase
Methotrexate, TMP-SMX:Inhibit DHF reductase
phenytoin: Inhibits intestinal conjugase
OCP, ETOH: Inhibits uptake of monoglutamate in jejunum
ETOH also inhibits the release of folate from the liver
S/S of folate deficiency
Megaloblastic anemia - weakness, etc
Low serum folate, incr serum homocysteine
NO neuro Sx
How should you Tx Megaloblastic anemia
Asses for vit B12 deficiency first, b/c folate will correct hematologic findings in both B12 and Folate deficiency, but NOT the neuro Sx of B12 deficiency
Stimuli for EPO
Hypoxemia, left-shifted OBC, high altitiude
Where is EPO synthesized
Endothelial cells of the peritubular capillaries
Reticulocyte count
Measure of effective erythropoiesis (bone marrow response to anemia); correct for the degree of anemia
Why need a corrected reticulocyte count

B/c it is fasely elevated in anemia

corrected = (actual Hct / 45) x reticulocyte count

> 3% = effective eryropoiesis i.e hemolytic anemia, after Tx of iron deficiency anemia with iron

<3% ineffecive i.e. aplastic anemia, untx iron deficiency anemia
What does HbF do to the OBC and what is the result
Shifts OBC to the left - causes the release of EPO - causes an incr in Hb, HCT, RBCs
Lab values in Thalasemia
Decr: Hb and Hct
Incr: RBC
What is the O2 and arterial PO2 in anemia
What 2 anemias have a change in the MCHC
Decr MCHC: Microcytic anemias
Incr MCHC: Hereditary sphirocytosis
Which anemia has an incr RDW? why?
Iron deficiency
B/c of mixture of normocytic and microcytic RBCs
What is the end product of heme degradation in macrophages
Unconjugated bilirubin
What are the Normocytic anemia High correct reticulocyte count (>3%), Extrinsic RBC defect
Micoangiopathic hemolytic anemia
In what type of anemia is serum ferritin (primary iron storage protein) decreased
Dx of iron deficiency anemia
In what 2 Dzs is serum ferritin (primary iron storage protein) increased
Anemia of chronic Dz

Iron overload
In what 2 Dzs is serum iron (iron bound to transferrin) decreased
Iron deficiency anemia (also characterized by incr tansferrin and thus incr TIBC)

Anemia of chronic Dz (also characterized by decr transferrin, and thus decr TIBC)
In what Dz is serum iron increased
Iron overload Dz (also charct by decr transferrin, thus decr TIBC)
What is the relationship b/w serum ferritin stores and transferrin (and thus TIBC)
Incr in serum ferritin stores causes decr synthesis of transferrin (and thus TIBC) = Anemia of chronic Dz and Iron overload

Decr ferritin stores casue and incr syn of transferrin (and thus TIBC) = iron deficiency anemia
DF iron sat and when is it increased and decr
% of binding sites on transferrin occupied by iron

Incr iron sat: iron overload Dz

Decr: Anemia of chronic Dz
iron deficiency anemia
Plummer-Vinson syndrome is associated with what type of anemia?

Characteristics of plummer vinson?
Chronic iron deficiency anemia
Characterized by:
Esophageal web (dysphagia for solids but NOT liquids)

At incr risk of squamous cell CA of the esophagus
What is the electrophoresis pattern in alpha thal trait
Normal - all Hb types require the alpha chain
Hbs found in Beta Thal major
Incr: HbA2 and HbF
Pathogenesis of sideroblastic anemia?

3 Main causes of sideroblastic anemia?
Defect in heme synthesis in the mito, iron accumulates in the mito forming ringed siderblasts

1. Chronic ETOH (MCC)

2. Pyridoxine deficiency - INH therapy; B6 is a cofactor for ALA synthase

3. Pb poisoning - Denatures enzymes:
a. Ferrochelatase - Iron can't bind with protoporphyrin to form Heme = incr protoporphyrin
b. ALA dehydrase = incr ALA
c. Ribonuclease - Ribosomes can't be degraded = coarse basophilic stippling
Clinical sx of Pb poisoning
1. Colicky abd pain w/diarrhea
2. Neuron damage and demylination
3. Growth retardation
4. Pb deposition in epiphysis
5. Peripheral neuropathy in adults - footdrop due to peroneal palsy
6. Fanconi's syndrome
MCC of folate deficiency
What lab value will be increased in both folate and Vit B12 deficiency
and what blood cell is characteristic
NOTE: Folate deficiency is the MCC of incr homocysteine levels

Hypersegmented PMNs
Most sensitive test for vit B12 deficiency
Incr. Methylmalonic acid
Type of anemia found in CRF? Why?
What type of cell is often present?
Normocytic anemia MC due to decr syn of EPO

Burr cells = RBCs with undulating membrane
MC viral cause of an aplastic crisis
Parvovirus B19
Hereditary elliptocytosis
Decfect in spectrin and band 4.1

Associated with none to mild hemolytic anemia

Lab: Ellipocytes >25% of RBCs in peripheral blood
Incr osmotic fragility
Sreening and confirmatory test for PNH
screeing test: Sucrose hemolysis test - sucrose enhances compliment destruction of RBCs

Confimatory test: Acidified serum - acidified serum activates the alternative pathway causing hemolysis
PNH results in increased risk of what two things
Increased risk of vessel thrombosis (heptaic vein) and for developing AML
MC clinical finding of HbSS in infants?
Dactylitis - painful swelling of hands and fet in infants (6-9mo) due to bone infarcts
Renal findings in HbAS (also in HbSS)
Sickling occurs in peritubullar capillaries of the medulla due to low O2 tention - presents with microhematuria due to infarction
Inheritance, pathogenesis, and clinical findings, and lab findings of Pyruvate kinase deficiency
Intrinsic defect, extravascular hemolysis

Chronic lack of ATP leads to membrane damage; also have incr in 2,3 BPG, which ends up Right shifting OBC, therefore offsets clinical effects of anemia

Lab: RBCs with thorny projections (echinocytes)
Cause and finding of macroangiopathic hemolytic anemia
Hemolysis caused by valvular defects - Ao valvular stenosis

Extrinsic defect with intravascular hemolysis

Schistocytes in peripheral blood