Hematology

What is the term for a decrease in RBCs and hgb resulting in decreased oxygen delivery to the tissues?

What lab finding is when anemia is suspected?

What causes a decreased delivery of RBCs into circulation?

What causes an increased loss of RBCs from the circulation?

What is the most common form of anemia in the US?

What clinical condition is consistent with the findings:
Microcytic/hypochromic anemia
Decreased serum iron, ferritin, hgb, HCT, RBC, retic
Increased RDW, TIBC
Ovalocytes/Elliptocytes

What clinical condition is consistent with the findings:
Normocytic/normochromic anemia or slightly microcytic/hypochromic anemia
Increased ESR
Normal-Increased Ferritin
Decreased serum iron and TIBC
Inability to use available iron for hemoglobin production 
Has an impaired release of storage iron associated with increased hepcidin levels

What cells show up when there is excess iron accumulating in the mitochondrial region of the immature erythrocyte in the bone marrow and encircles the nucleus?

What stain is used to see siderocytes?

Name the 2 types of sideroblastic anemia

What clinical condition is consistent with the findings:
Microcytic/hypochromic anemia
Increase ferritin and serum iron
TIBC decreased
Caused by blocks in the protoporphyrin pathway resulting in defective hemoglobin synthesis and iron overload

What clinical condition is consistent with the findings:
Normocytic/normochromic anemia
Coarse basophilic stippling
Has multiple blocks in the protoporhyrin pathway that affects heme synthesis

What clinical condition are a group of inherited disorders characterized by a block in the protoporphyrin pathway of heme synthesis?

What clinical condition is consistent with the findings:
Pancytopenia
Macrocytic/normochromic anemia
Oval macrocytes and tear drop cells
Hypersegmented neutrophils
Inclusions of Howell Jolly, NRBCs, basophilic stippling, pappenheimer bodies, and cabot rings
Increased LD, bilirubin, and iron
Megaloblastic cells in blood and bone marrow
Defective DNA synthesis that causes abnormal nuclear maturation and the nucleus matures slower than the cytoplasm (asynchronism)

Name the causes of of megaloblastic anemias

What clinical condition is caused by deficiency of intrinsic factor, antibodies to intrinsic factor, or antibodies to parietal cells?

What are the causes of vitamin B12 deficiency?

What are the causes of folic acid deficiency?

Name what is included in non-megaloblastic macrocytic anemias

What clinical condition:
Bone marrow failure causes pancytopenia
Labs: Decreased H&H and retics, normocytic/normochromic
No response to erythropoietin

What is the name of the genetic aplastic anemia, that is an autosomal recessive trait?

What clinical condition is a true red cell aplasia (WBCs and plts are normal)?

What clinical condition:
Hypoproliferative anemia caused by replaement of bone marrow hematopoietic cells by malignant cells or fibrotic tissue
Labs: Normocytic/normochromic, leukoerythroblastic blood picture

What clinical condition:
Sudden blood loss
Normocytic/normochromic anemia
Initially normal retic and H&H, then in a couple of hours drop in H&H, increase in plts, leukocytosis with left shift
Reticulocytosis in 3-5 days

What clinical condition:
Gradual long-term loss of blood
Initially normocytic/normochromic, that over time causes a decrease in H&H
Gradual loss of iron, causing microcytic/hypochromic anemia

What clinical condition is:
The most common membrane defect of RBCs
Is due to increased permeability of the membrane to sodium
Had decreased surface area to volume ratio Caused by a mebrane defect due to abnormal permeability to both Na and K - causing RBCs to swell
Spherocytes 
Increased osmotic fragility
Increased serum bilirubin

What clinical condition has an increased cholesterol:lecithin ratio in the membrane due to abnormal plasma lipid concentrations?

What clinical condition:
Sex linked enzyme deficiency
Most common enzyme deficiency in the hexose monophosphate shunt
Result in oxidation of hemoglobin to methemoglobin (Fe³⁺)
Denatures to form Heinz bodies

What clinical condition:
The most common enzyme deficiency in Embden-Meyerhof pathway
Severe hemolytic anemia with retics and echinocytes

What clinical condition:
Is an acquired membrane defect in which the RBC membrane has an increased sensitivity for complement binding as compared to normal RBCs
Pancytopenia
Chronic intravascular hemolysis causes hemoglobinuria and hemosiderinuria at an acid pH at night
Low LAP score
Ham's and sugar water tests used in diagnosis
Increased incidence of acute leukemia

What clinical condition:
Have RBCs costed with IgG and/or complement
Labs: Spherocytes, increased osmotic fragility, increased bilirubin, increased retic, occasional NRBC, DAT POS

What clinical condition:
Have RBCs coated with IgM and complement at temps below 37 degrees celcius
Labs: Seasonal symptoms, RBC clumping can be seen micro-and macroscopically, MCHC >37 g/dL, increased bilirubin, increased retic, DAT Positive

What clinical condition has:
An IgG biphasic Donath-Landsteiner antibody with P specificity fixes complement to RBCs in the cold
Complement coated RBCs lyse when warmed to 37 degrees celcius
Increased bilirubin and hemoglobin
Decreased haptoglobin
Donath-Lansteinter positive

What clinical condition is:
ABO incompatibility causes an immediate reaction with massive intravascular hemolysis that is complement coated
Usually IgM antibodies
Can trigger DIC due to release of tissue factro from the lysed RBCs
DAT positive
Increase hemoglobin

What clinical condition:
May be due to Rh incompatibility (erythroblastosis fetalis)
- Severe anemia, NRBCs, DAT positive,
- Increased bilirubin that causes kernicterus leading to brain damage
May be due to ABO incompatibility
- Group O women develops IgG antibody that crosses the placenta and coats fetal RBCs when fetus is group A or B
- Mild or no anemia, few spherocytes

What clinical condition is:
Systemic clotting is initiated by activation of the coagulation cascade due to toxins or conditions that trigger release of procoagulants
Multiple organ failure can occur due to clotting
Fibrin is deposited in small vessels, causing RBC fragmentation

What clinical condition:
Occurs most often in children following a gastrointestinal infection
Clots form, causing renal damage

What clinical condition:
Likely due to a deficiency of the enzyme ADAMTS 13 that is responsible for breaking down large von Willebrand factor multimers
When multimers are not broken down, clots form, causing RBC fragmentation (schistocytes) and CNS impairment

Name the most common (in order) amino acid subsitutions disorders

What clinical condition:
Caused when valine replaces glutamic acid at position 6 of both beta chains
No Hgb A produced
Causes tissue necrosis
All organs affected - kidney failure is the most common
Vaso-occlusive crisis occurs with increased bone marrow response to the hemolytic anemia
Severe normochromic/normocytic anemia
Polychromasia (result of retics), Sickle cells, target cells, NRBCs, pappenheimer bodies, howell-jolly bodies
M:E ratio decreases
Increased bilirubin
Decreased haptoglobin

What is the amino acid substitution for Sickle cell?

What clinical condition is caused when valine replaces glutamic acid at position 6 on one beta chain?

What clinical condition:
Caused when lysine replaces glutamic acid at position 6 on both beta chains
No Hgb A produced
Normochromic/normocytic anemia with target cells
Characterized by intracellular rodlike C crystals

What is the amino acid substitution of Hgb C Disease?

What clinical condition:
Is a double heterozygous condition where abnormal sickle gene from one parent and an abnormal C gene from the other parent is inherited
Moderate to severe normocytic/normochromic anemia
Target cells, charcteristic SC crystals, rare sickle cells or C crystals
Positive hemoglobin solubility screening test

What clinical condition is caused when lysine replaces glutamic acid at position 26 on the beta chain?

What clinical condition is caused when glycine replaces glutamic acid at position 121 on the beta chain?

What types of conditions are included in Thalassemia major?

What types of conditions are included in Thalassemia minor/trait?

What clinical condition:
Markedly decreased rate of synthesis or absence of both beta chains results in excess of alpha chains
No Hgb A can be produced
Compensate with up to 90% Hgb F
Severe microcytic/hypochromic anemia
Target cells, many NRBCs, basophilic stippling, howell-jolly bodies, pappenheimer bodies, heinz bodies
Increased serum iron and bilirubin

What clinical condition:
Decreased rate of synthesis of one of the beta chains, other beta chain is normal
Mild microcytic/hypochromic anemia
Normal or slightly increased RBC count
Target cells, basophilic stippling
Hgb A is slightly decreased, but Hgb A₂ is slightly increased to compensate

What clinical condition:
All 4 alpha chains are deleted
No normal hemoglobins are produced
80% hemoglobin Bart's (gamms₄) produced
Cannot carry oxygen
Incompatible with life
Die in utero or shortly after birth

What clinical condition:
3 alpha chains are deleted - leads beta chain excess
Hemoglobin H (beta₄) - an unstable hemoglobin is produced
Moderate microcytic/hypochromic anemia
Heinz bodies form and are destroyed in the spleen

What clinical condition:
2 Alpha chains are deleted
Patients are usually asympomtatic and discovered accidently
Up to 6% Hgb Bart's in newborns
Mild microcytic/hypochromic anemia
Hight RBC count
Target cells