Goljan Hematology Rbc Anemia

Front 1

What are normocytic anemias with a corrected Reticulocytes count below 3%

Back 1

acute blood loss, early iron deficiency or anemia of chronic disease, aplastic anemia, and chronic renal failure

Front 2

How many days does it take to see a reticulocyte response to acute blood loss?

Back 2

5-7 days

Front 3

how to distinguish between early anemia of chronic disease and early iron deficiency?

Back 3

serum ferritin:
anemia of chronic inflation= high serum ferratin
iron deficiency= low serum ferratin

Front 4

What are causes of aplastic anemia?

Back 4

50-70% idiopathic, drugs/toxins: chloramphenicol, alkylating agents, benzen, DDT, parathion, infection: EBV,CMV, parvovirus, nonA nonB hepatitis, whole body radiation, thymoma, paraoxysmal nocturnal hemoglobin uria

Front 5

What is the clinical presentation with aplastic anemia?

Back 5

normocytic anemia, neutropenia (infection/fever), bleeding/thrombocytopenia, pancytopenia, reticulocytopenia

Front 6

What is the pathogeneis of aplastic anemia?

Back 6

Either antigenic alteration of multipotent myeloid stem cells or defective/deficient stem cells (acquired or hereditary)

Front 7

What is the bone marrow like in aplastic anemia?

Back 7

Hypocellular bone marrow

Front 8

What is the most common cause of anemia with chronic renal failure?

Back 8

Decreased synthesis of EPO

Front 9

lab findings in chronic renal failure anemia?

Back 9

normocytic anemia, burr cells, prolonged bleeding due to defect in platelet agggregation, thrombocyopenia

Front 10

Examples of intrinsic hemolytic anemias

Back 10

G6PD defienciency, membrane defects, abnormal hemoglobin

Front 11

Normocytic anemias with corrected reticulocyte count >3%

Back 11

intrinsic or extrinsic hemolytic anemias

Front 12

extrinsic causes for RBC hemolysis

Back 12

stenotic aortic valve, immune destruction, phagocytosis in the splean

Front 13

signs of intravascular hemolysis

Back 13

increase plasma/urine HB, hemosiderinuria, decreased serum haptoglobin, increased serum LDH

Front 14

2 sites where extravascular hemolysis occurs

Back 14

spleen & liver

Front 15

clinical in hereditary spherocytosis

Back 15

increased bilirubin (jaundice), splenomegaly, aplastic crisis esp children after infection, increased # bile stones

Front 16

Pathogenesis of hereditary spherocytosis

Back 16

autosomal dominant disorder that results in loss of RBC membrane causing spheroctye formation and extravascular hemolysis in spleen

Front 17

Type of mutation in hereditary spherocytosis

Back 17

Most common: ankyrin, also band 2, spectrin, or band three

Front 18

lab findings in hereditary spherocytosis

Back 18

normocytic anemia >3% corrected retics, increased MCHC, increased RBC fragility (rupture hypotinic solution)

Front 19

hereditary elliptocytosis

Back 19

autosomal dominant defect in spectrin and band caused splenomegaly, no or mild hemolytic anemia, with increased fragilityand 25%+elliptocytes

Front 20

tx of hereditary elliptocytosis

Back 20

splenectomy if symptomatic

Front 21

Defect in paroxysmal nocturnal hemoglobinuria?

Back 21

acquired membrane defect that causes loss of anchor for DECAY ACCELERATION FACTOR

Front 22

Why does paroxysmal nocturnal hemoglobinuria occur at night?

Back 22

resp. acidosis enhances intravascular completement mediated lysis of neuts, RBCs and platelets

Front 23

tests for paroxysmal nocturnal hemoglobinuria

Back 23

sucrose hemolysis test, acidified serum test, norcyotic anemia with pancytopenia (microcytic if Fe def. develops), decreased serum hatpoglobun, increased urine Hb

Front 24

Cause of sickling?

Back 24

missense point mutation (valine for glutamic acid) in beta chain

Front 25

factors that increase likelihood of sickling?

Back 25

HbS concentration >60%, acidosis, volume deplation, hypoxemia

Front 26

Is sickling irreversible?

Back 26

Only initially with O2

Front 27

Complication of sickling?

Back 27

microvascular occlusions causing ischemia damage

Front 28

2 things that help prevent sickling

Back 28

increased HbF (prevents sickling until 5-6 months), hydroxyurea

Front 29

Clinical findings in sickle cell

Back 29

dactylitis, vaso-occlusion of pulmonary capillaries, chest pain, aseptic necrosis femoral head, autosplenectomy

Front 30

Cause of renal problems in sickle carriers?

Back 30

low o2 in renal medulla, sickling that can cause renal papillary necrosis and microhematuria

Front 31

lab findings in sickle cell?

Back 31

blood smear-sickle and target cells

Front 32

what is the screening for sickle cell?

Back 32

sodium metabisulfite causes sickling by reducing O2 or Hb electrophoresis

Front 33

populations with greatest risk for G6PD deficiency?

Back 33

Black American, Greeks, Italian (mediterranian more severe and chronic)

Front 34

type of hemolysis in G6PD def?

Back 34

mostly intravascular with some extravascular

Front 35

problem with G6PD def?

Back 35

not enough NADPH and GSH (gluathion) suspetible to oxidative stress (esp in infection)

Front 36

smear finding in G6PD def?

Back 36

Heinz body

Front 37

Clinical findings in G6PD def?

Back 37

sudden back pain 2-3 days after oxidative stress or drugs such as primaquine, chloroquine, dapson, sulfa and Fava beans

Front 38

test for G6PD def?

Back 38

screen during hemolysis for heinz bodies with supravital stain, after crisis RBC enzyme analysis to confirm

Front 39

Pyruvate Kinase deficiency

Back 39

autosomal recessive disease causing chronic lack of ATP resulting in membrane damage

Front 40

clinical findings in Pyruvate Kinase deficiency

Back 40

hemolytic anemia with jaundice at birth, increase 2,3 BPG, RBCS with thorny projections

Front 41

test for pyruvate kinase deficiency

Back 41

RBC enzyme assay

Front 42

most common cause of autoimmune hemolytic anemias?

Back 42

lupus (more common in women than men)

Front 43

what are warm type of autoimmune hemolytic anemias?

Back 43

IgG antibodies (70% of AIHA)

Front 44

what are the antibodies in cold type?

Back 44

IgM antibodies (30% of AIHA)

Front 45

what are drugs that can induce autoimmune hemolytic anemias?

Back 45

penicillin(type II hypersensitivity), quinidine (type III), and methyl dopa

Front 46

clinical findings in autoimmune hemolytic anemia?

Back 46

jaundice, raynauds in cold, hepatosplenomegaly

Front 47

lab findings in autoimmune hemolytic anemia?

Back 47

+ direct combs test (detects RBCS with IgG or C3b), indirect coombs, hemoglobinuria, decreased haptoglobin

Front 48

smear findings in autoimmune hemolytic anemia?

Back 48

normocytic anemia, spherocytosis

Front 49

causes of microangiopathic anemia?

Back 49

platelet thrombi (hemolytic uremia syn, thrombotic thrombocytopenic purpura),& Fibrin thrombi (DIC, HELLP syndrome)

Front 50

causes of macroangiopathic anemia?

Back 50

aortic stensis or prosthetic heart valves

Front 51

lab findings in angiopathic hemolytic anemias?

Back 51

normocytic, hemoglobinuria (if long enoough can cause Fe def), decreased haptoglobin, schistocytes in peripheral blood

Front 52

Malaria is caused by what type of misquito?

Back 52

female anopheles misquito

Front 53

clinical findings in malaria?

Back 53

fever when RBCs rupture intravascularly (prominent over extrasvascular), splenomegaly

Front 54

smears in malaria?

Back 54

organisms in RBC

Front 55

3 types of plasmodium and fever patterns?

Back 55

plasmodium vivax-48 hours
plasmodium falciprium-daily spikes NO pattern
plasmodium malariae-72 hours