Anemia - Ch. 14 Flashcards Preview

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Flashcards in Anemia - Ch. 14 Deck (67):
1

Pale, weak, easily fatigued, exercise intolerance

Anemia

2

After 3-5 days...reticulocytosis, leukocytosis, thrombocytosis, erythroid precursor cells (BM) w/ blue-red cytoplasm, increased EPO

Anemia of acute blood loss

3

Anemia, splenomegaly, unconjugated jaundice, decreased haptoglobin

Extravascular hemolytic anemia

4

Anemia, hemoglobinemia, hemoglobinuria, hemosiderinuria, unconjugated jaundice, red-brown urine, decreased haptoglobin

Intravascular hemolytic anemia

5

BM - normoblasts (erythroid precursors)
PB - reticulocytosis, hemosiderosis
Labs - increased EPO, increased degradation products

Hemolytic anemia

6

RUQ abdominal pain, hemolytic anemia

Indicate what?

What else tends to happen in this setting?

Pigment gallstones

CHRONIC hemolytic anemia

Splenomegaly

7

Caucasian, anemia, splenomegaly, jaundice, episode of worsened anemia symptoms during an infection

Hereditary spherocytosis - complicated by aplastic crisis (parvovirus infection)

8

Osmotic lysis in hypotonic salt solutions

Hereditary spherocytosis

9

Heinz bodies, bite cells, spherocytes

G6PD deficiency (the Heinz bodies cause the formation of bite cells and spherocytes)

10

Episodic anemia, jaundice, weakness, pallor. Black or Mediterranean male. No splenomegaly or gallstones.

G6PD deficiency

11

Caucasian teenager, anemia, splenomegaly, jaundice, episode of cervical lymphadenopathy and swollen tonsils w/ decreased haptoglobin

Hereditary spherocytosis - complicated by hemolytic crisis (infectious mononucleosis)

12

Sickle cell, rapid splenic enlargement, hypovolemia, shock

Sequestration crisis

13

Crewcut X-Ray, enlarged cheekbones

- Sickle cell disease
- Beta-thalassemia major

14

Beta-4 tetramers

Hemoglobin H - alpha-thalassemia - adolescents and adults

15

Gamma-4 tetramers

Hemoglobin Barts - alpha-thalassemia - young kids

16

Genetic differences in alpha vs beta thalassemias

Alpha - gene deletions
Beta - point mutations

17

Asians vs blacks - alpha-thalassemia genotypes

Asian - a/a -/-
Black - a/- a/-

18

HbH disease - explain

3 alpha-globin mutations = HbH formation = extreme tissue hypoxia, intracellular inclusions = red cell sequestration = moderate anemia

19

Hydrops fetalis - explain

Symptoms

4 beta-globin mutations = Hg barts formation + embryonic Hb tetramer formation

Pallor, genralized edema, massive HSM

20

Which thalassemias require regular blood transfusions?

Beta-major, hydrops fetalis

21

Intravascular hemolysis (anemia, jaundice, hemoglobinemia, hemoglobinuria, hemosiderin), autoimmune disease, venous thrombosis

Future consideration?

Paroxysmal nocturnal hemoglobinuria

Transform in AML or MDS (since its an HSC)

22

Immunohemolytic anemia - what is it?

Hemolysis type?

Premature destruction of RBCs due to antibodies or complement that attach

Extravascular (usually)

23

Causes of Warm Antibody Type immunohemolytic anemia

Antibody type? Against what?

- Idiopathic
- SLE
- Drugs - penicillin, cephalosporins, alpha-methyldopa
- Lymphoid neoplasms

IgG - anti-Rh

24

Warm Antibody immunohemolytic anemia - what happens?

IgG antibodies bind RBCs, causing (usually) extravascular hemolysis (loss of membrane --> spherocytes --> sequestration --> splenomegaly

25

Causes of Cold Agglutinin Type immunohemolytic anemia

Antibody type?

- Acute = Mycoplasma, EBV (mono)
- Chronic = idiopathic, lymphoid neoplasms

IgM (M = Mycoplasma)

26

Cold Agglutinin immunohemolytic anemia - what happens?

Child has self-limited infection OR chronic lymphoid neoplasm OR no identified cause --> RBCs circulate to COLD areas, IgM binds --> circulate to WARM areas, IgM releases --> C3b deposits cause extravascular hemolysis

27

Besides hemolytic anemia, what else is seen in Cold Agglutinin immunohemolytic anemia?

Vascular obstruction in cold extremities --> pallor, cyanosis, Raynaud phenomenon

28

Cold Hemolysis immunohemolytic anemia - cause?

Antibody type? Against what?

Child after viral infection

IgG - binds to P blood group

29

Cold Hemolysis immunohemolytic anemia - what happens?

Viral infection causes IgG autoantibodies --> RBCs circulate to COLD areas, IgG binds --> circulate to WARM areas, IgG causes complement-mediated lysis

30

Causes of trauma to RBCs that lead to hemolytic anemia

- Prosthetic cardiac valves
- Microangiopathies (DIC, TTP, HUS, malignant HTN, SLE, cancer)

31

Schistocytes, burr cells, helmet cells, triangle cells

Trauma-induced hemolytic anemia

32

Abnormally large erythroid precursors (promegaloblasts), giant metamyelocytes, and band forms in the BM

Megaloblastic anemia

33

Abnormally large RBCs and hypersegmented neutrophils in peripheral blood

Megaloblastic anemia

34

Vegans and vegetarians (B12 or folate)

B12 deficiency

35

Fish tapeworm (B12 or folate)

B12 deficiency

36

Chronic alcoholic (B12 or folate)

Folate deficiency

37

Pregnancy, infancy, hemolytic anemia (B12 or folate)

Folate deficiency

38

Hemodialysis (B12 or folate)

Folate deficiency

39

Oral contraceptives (B12 or folate)

Folate deficiency

40

Why the neuro symptoms in B12 deficiency?

Increased methylmalonate --> abnormal fatty acid accumulation in neuron lipids --> myelin breakdown

41

Initiating event to autoimmune gastritis and pernicious anemia

Autoreactive T-cell response against gastric mucosa, causing autoantibody formation against intrinsic factor

42

Pernicious anemia is associated w/ what else?

Thyroiditis, adrenalitis

43

Non-pernicious causes of B12 deficiency

- Achlorhydia (ex. patient taking HCl supplement)
- Gastrectomy
- Exocrine pancreas loss (can't split B12-haptocorrin)
- Ileal resection or disease
- Fish tapeworm
- Pregnancy, hyperthyroid, cancer, chronic infection (all increased requirement)

44

Intestinalization, beefy glazed tongue, decreased HCl production, spastic paraparesis, paresthesias

Pernicious anemia

45

Pernicious anemia...what to be cautious of?

Gastric carcinoma and atherosclerosis risks

46

Elevated homocysteine, normal methylmalonate

Folate deficiency

47

TMPRSS6 mutation

Elevated hepcidin --> microcytic iron-deficient anemia

48

A patient is iron deficient, but has no dietary issues, no GI issues, no bleeding issues, and seems perfectly healthy. She does not respond to iron supplementation. Potential explanation?

TMPRSS6 mutation

49

Abnormally low hepcidin...explanation?

Hemochromatosis (primary or secondary)

50

Secondary hemochromatosis...major cause?

Ineffective hematopoiesis --> decreased hepcidin

51

Decreased transferrin saturation, decreased serum ferritin

Iron deficiency

52

Spooning of nails, hair loss, malabsorption, atrophic tongue

Iron deficiency

53

Patient craving ice chips, soil, clay, or paper

Pica - iron deficiency

54

Smooth glazed tongue, trouble swallowing foods, microcytic anemia

Plummer-Vinson syndrome

55

Increased PIBC

Iron deficiency

56

Low serum iron, reduced TIBC, increased hemosiderin

Anemia of chronic disease

57

Describe anemia of chronic disease

Patient has source of chronic systemic inflammation, causing increased IL-6, which causes increased hepcidin, which causes iron-starved erythroblasts despite having plenty in marrow macrophages. Hepcidin also inhibits EPO production.

58

Causes of ACD

- Osteomyelitis, bacterial endocarditis, lung abscess
- RA, regional enteritis
- Carcinomas, Hodgkins lymphoma

59

Baby with pancytopenia, kidney hypoplasia, spleen and bone anomalies...what is it? Cause?

Fanconi anemia - defective DNA repair

60

Causes of aplastic anemia

Chemo, benzene, chloramphenicol, gold salts, viral infections, non-hepatitis hepatitis, irradiation, other

61

How does chemo, radiation, etc. cause aplastic anemia?

Antigenic alteration of HSCs by the agent --> T-HELPER CELL suppression/destruction of HSCs via IFN-GAMMA and TNF

62

Anemia, decreased Hb and Hct, normal WBCs and platelets, lack of erythroid progenitors in the BM

Associated with what?

Pure red cell aplasia

Thymoma, LGL leukemia, drugs, autoimmune disorders, parvovirus (aplastic crises)

63

Myelophthisic anemia

Most common causes? (3)

BM failure due to space-occupying lesions that replace normal marrow components

Metastatic carcinomas, granulomatous disease, MPD spent phase

64

Leukoerythroblastosis, dacrylocytic cells

Marrow replacement of some kind

65

Effect of uremia on RBCs

Diminished EPO synthesis --> anemia

66

Hypothyroidism + anemia...what kind?

Normochromic, normocytic

67

Hepatocellular liver disease + anemia...why?

Lipid abnormalities --> RBC membranes acquire phospholipid and cholesterol --> destruction