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HemeOnc > Pathophys: Hematology > Flashcards

Pathophys: Hematology Flashcards

1
Q

hemoglobinopathy

A

group of disorders (inherited) = abnormal production or structure of hemoglobin molecule (e.g., sickle cell anemia)

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2
Q

coagulopathy

A

a disease/condition affecting the bloods ability to coagulate

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3
Q

aplasia

A

incomplete, retarded or defective development of tissue/organ; cessation of the usual regenerative process in a tissue/organ [aplastic anemia: body stops producing new blood cells]

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4
Q

anisocytosis

A

a variation in size of cells (esp RBCs, pernicious anemia)

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5
Q

anemia

A

hemoglobin concentration is below normal bc of a deficiency in RBCs and/or a low level of hemoglobin in cells

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6
Q

hemolysis/hemolytic

A

break down of RBCs

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7
Q

polycythemia

A

disorder of bone marrow (resulting in over production of RBCs, and a little increase in WBCs and platelets)

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8
Q

cytosis vs. cytopenia

A

cytois - condition in which there is more than the usual number of cells
cytopenia - deficiency of cellular elements of the blood

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9
Q

poikilocytosis

A

a condition characterized by poikilocytes in the blood; a poikilocyte is an abnormally shaped RBC

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10
Q

pancytopenia

A

abnormal reduction in the number of erythrocytes, WBCs and platelets in the blood (all 3 must be low)

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11
Q 
mycrocytic anemias (2)
(5 questions)
A

iron deficiency

thalassemia - major/mino

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12
Q 
macrocytic anemias (4)
(4 questions)
A

folic acid deficiency
B12 deficiency
liver disease
myelodysplasia

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13
Q 
normocytic anemia (1)
(4 questions)
A

anemia of chronic disease

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14
Q 
hemolytic anemia (7)
(4 questions)
A 
1 sickle cell anemia (Hb S disease)
2 sickle cell trait
3 G6PD deficiency
4 cold agglutinin disease
5 hemoglobin S-C disease
6 hereditary sperocytosis
7 autoimmune hemolytic anemia
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15
Q

what is hemochromatosis?

1 question

A

disease in which the body absorbs too MUCH iron from diet; iron is stored in liver and pancreas and can be toxic leading to cancer, heart arrhythmias and cirrhosis

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16
Q

bone marrow failure diseases (3)

1 question

A

aplastic anemia
pure red cell aplasia
myelodysplasia

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17
Q

leukemias and myeloproliferative disorders (7)

6 questions

A
  1. acute lymphocytic leukemia (ALL)
  2. chronic lymphocytic leukemia (CLL)
  3. polycythemia vera
  4. myelofibrosis
  5. acute myelogenous leukemia (AML)
  6. chronic myelogenous leukemia (CML)
  7. essential thrombocytosis
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18
Q

lymphomas and plasma cell dyscrasias (4)

6 questions

A
  1. hodgkin’s disease
  2. multiple myeloma
  3. non-hodgkins lymphoma
  4. burkitts lymphoma
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19
Q

disorders of hemostasis (5)

3 questions

A
  1. immune thrombocytopenic purpura
  2. thrombotic thrombocytopenic purpura
  3. hemolytic-uremic syndrome
  4. henoch-schonlein purpura
  5. heparin-induced thrombocytopenia
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20
Q

hereditary disorders of coagulation (3)

2 questions

A
  1. factor VIII deficiency (hemophilia A)
  2. factor IX deficiency (hemophilia B)
  3. von Willebrands disease
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21
Q

acquired disorders of coagulation (4)

2 questions

A
  1. vitamin K deficiency
  2. coagulopathy of liver disease
  3. disseminated intravascular coagulation (DIC)
  4. acquired platelet disorders
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22
Q

acquired and congenital hypercoaguable states (6)

2 questions

A
  1. factor V Leiden
  2. malignancy
  3. activated protein C resistance
  4. lupus anticoagulant
  5. protein C deficiency
  6. protein S deficiency
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23
Q

iron deficiency

A
  • blood loss until proven otherwise
  • microcytic
  • smooth tongue, brittle nails, koilonychias
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24
Q

thalassemia

A
  • defect making hemoglobin (genetic)
  • greater degree of microcytosis
  • normal or elevated RBC and retic (unique of the anemias)
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25
Q

folic acid deficiency

A
  • NO NEURO defects
  • macrocytic
  • glossitis
  • pallor
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26
Q

B12 deficiency

A
  • YES NEURO DEFECTS
  • macrocytic
  • pallor
  • glossitis
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27
Q

liver disease

A
  • liver enzymes (which transport B12) not being produced
  • B12 not absorbed
  • macrocytic
28
Q

anemia of chronic disease

A

-chronic disease associated with it

29
Q

sickle cell anemia

A
  • hemoglobin S, homozygous, hereditary
  • recurrent pain
  • transfusion, folic acid
30
Q

sickle cell trait

A
  • hemoglobin S, heterozygous, hereditary
  • no tx, genetic counsiling
  • increased risk
31
Q

G6PD deficiency

A
  • enzyme defect
  • HEINZ bodies! cause membrane damage
  • episodic hemolytic anemia
  • avoid antioxidant drugs
32
Q

cold agglutinin disease

A
  • ACQUIRED
  • IgM “cold” agglutinin
  • anemia with exposure to cold
  • lysis is intravascular
  • coombs + test
  • tx w/ rituximab
33
Q

hemoglobin SC disease

A
  • no sickling
  • hemoglobin S and C (heterozygous)
  • mild course
34
Q

autoimmune hemolytic anemia

A
  • acquired
  • IgG “warm” autoantibody
  • rapid onset of life threatening anemia
  • spherocytes!
  • coombs + test
  • tx w/ steroids and maybe splenectomy
35
Q

aplastic anemia

A
  • hematopoetic stem cell
  • bone marrow failure
  • pancytopenia
  • pupura, petichiae, fatigue, mucosal bleed
  • NO ABNORMAL CELL MORPH
  • NO LYMPH, LIVER, SPLEEN issues
36
Q

pure red cell anemia

A

-autoimmune
-bone marrow failure
-low retic count, normal RBC
diff than aplastic (all cell lines)
diff than myelodysplasia (weird morphology)

37
Q

myelodysplasia

A
  • acquired
  • bone marrow failure
  • PRELEUKEMIA
  • MCV normal or increased (macrocytic anemia)
  • <20% blasts
  • MORPHOLOGIC abnormalities
38
Q

ALL

A

childhood disease
lymphocytes (b and t cells)
pancytopenia
>20% blasts

39
Q

AML

A

adult onset
myeloid cells
pancytopenia
>20% blasts

40
Q

CLL

A

B lymphocytes
lymphocytosis (lots!)
no tx unless symptomatic

41
Q

CML

A

PHILADELPHIA mutation!
increased WBC count
progress to AML

42
Q

polycemia vera

A

increased RBCs (and increased other cells too)
expanded blood volume
thrombosis
increased B12

43
Q

myelofibrosis

A

poikilocytosis
weird platelet morphology
other counts variable

44
Q

essential thrombocytosis

A

increased platelets
thrombosis
oral hydroxyurea

45
Q

hodgkins disease

A

young adult
lymphadenopathy
B cell lymphoma
rare

46
Q

multiple myeloma

A

plasma cell cancer

destruction of bone marrow and bone

47
Q

non hodgkins lymphoma

A

more common than HL
tumors from lymph tissue
B cell (85%)

48
Q

burkitts lymphoma

A

aggressive B cell NHL
rapid growth of tumors and lysis
high risk EBV + malaria
prevalent in children in Africa

49
Q

immune thrombocytopenic purpura

A

auto immune disease
destruction of platelets
can be seen w/ lupus, lymphoma, meds, hep C, HIV
“diagnosis of exclusion”

50
Q

thrombotic thrombocytopenic purpura

A 
thrombocytopenia 
destruction of platelets
fever
NEUROLOGIC S/S
ADULTS
schistocytes
51
Q

hemolytic-uremic syndrome

A 
thrombocytopenia
destruction of platelets
KIDNEY DISEASE, DIARRHEA
KIDS
schistocytes
52
Q

henoch-schonlein purpura

A 
vasculitis in children (arthritis, abd pain)
palpable purpura (extremities, buttocks)
53
Q

heparin induced thrombocytopenia

A

BOTH thrombocytopenic and prothrombic state (YAW!)

d/c heparin immediately

54
Q

factor 8 deficiency (hemophilia A)

A 
hereditary (more common than hemophilia B)
elevated PTT (intrinsic)
MALES
unexpected bleed w/ trauma/invasion
joint/soft tissue bleeds
55
Q

factor 9 deficiency (hemophilia B)

A 
hereditary (less common than hemophilia A)
elevated PTT (intrinsic)
MALES
unexpected bleed w/ trauma/invasion
joint/soft tissue bleeds
56
Q

von willebrands disease

A 
hereditary (most common!)
PTT elevated (intrinsic)
type 2 like hemophilia A but FEMALES AFFECTED
57
Q

vitamin K deficiency

A

acquired
elevated PT (extrinsic)
issue w/ diet/absorption/production

58
Q

coagulopathy of liver disease

A

acquired
decrease synthesis of clotting factors and fibrinogen
elevated PT (extrinsic) and PTT in advanced disease

59
Q

DIC

A 
acquired
decreased platelets and fibrinogen
elevated PT and PTT
secondary disorder
bleeding at mult. sites
60
Q

acquired platelet disorders

A

acquired
drugs increase thrombin time
heparin has antidote, newer drugs dont!

61
Q

activated protein C resistance

A

hereditary
most common inherited hypercoaguable state
VENOUS THROMBOSIS
act protein C (APC) is natural anticoagulant

62
Q

factor V leiden

A

hereditary
most common type of APC resistance
increased VENOUS thrombosis

63
Q

protein C deficiency

A

hereditary
activated protein C is natural anticoagulant
not enough C=too much clotting (VENOUS)

64
Q

protein S deficiency

A

hereditary
protein S is an activator of C
no S=no APC=VENOUS clots

65
Q

lupus anticoagulant

A

acquired
increased risk for ARTERIAL CLOTS
lupus anticoag is an PROTHROMBOTIC agent (misnamed anticoag)

66
Q

malignancy

A

acquired
most common cause of hypercoaguable state
VENOUS thrombosis

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