Path Exam 2 - Hematopathology

Front 1

Mean Corpusclar Volume (MCV)

Back 1

tells size of RBC in terms of cytoplasm, 80-96 fL, M:E ratio 3:1 (3x more WBCs than RBCs)

Front 2

ferritin

Back 2

storage pools that contain 20% of blood iron, 80% is found in hemoglobin, myoglobin, and iron contain enzymes

Front 3

transferrin

Back 3

transports iron in the plasma

Front 4

anemia

Back 4

a reduction of red cells

results from blood loss or failure or production (bone marrow disorder)

signs: pallor, fatigue, tachycardia

Front 5

iron deficiency anemia lab findings

Back 5

hypochromia (lighter color RBCs), polychromasia (pink hues), microcytosis (small RBCs, ↓MCV), ↓serum iron, ↓ferritin, ↑increase in total iron binding capacity

Front 6

pernicious anemia

Back 6

B12 deficiency

Front 7

normal B12 process

Back 7

1. B12 liberated from protein by pepsin
2. becomes bound to R-binder in saliva
3. R-B12 complex broken down by pancreatic enzymes
4. released B12 binds to IF produced by parietal cells
5. IF-B12 travels to ileum (IF receptors)
6. absorbed by mucosa and travels through blood by transcobalamin II

Front 8

pernicious anemia pathogenesis

Back 8

autoimmune destruction of mucosa, chronic gastritis

3 types of antibodies associated with: blocking B12-IF binding, block not permitting B12-IF absorption, parietal cell antibodies

Front 9

pernicious anemia diagnostic features

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moderate to severe megaloblastic anemia, leukopenia with hypersegemented granulocytes, mild to moderate thrombocytopenia, inability to absorb an oral dose of cobalamin

Front 10

hemolytic anemia

Back 10

premature destruction of red cells, accumulation of products of hemoglobic catabolism, compensatory increase in erythropoiesis within bone marrow

Front 11

intravascular hemolysis (hemolytic anemia)

Back 11

damage to red cells by mechanical immune or toxic facors

manifestations:
1. too much Hb in blood (hemoglobinemia)
2. Hb in urine (hemoglobinuria)
3. methalbumin in blood (methemalbuminemia)
4. jaundice
5. brown pigment in urine (hemosiderinuria)
6. decreases in serum haptoglobin (haptoglobin binds to free hemoglobin)

Front 12

extravascular hemolysis (hemolytic anemia)

Back 12

injured red cells, rendered foreign, or become less deformable are sequested by the spleen for destruction

manifestations:
1. anemia
2. jaundice
3. decrease in serum haptoglobin

Front 13

sickle cell anemia

Back 13

abnormality from mutation of ß-globulin gene (valine substituted for glutamate at 6th position of ß-globulin chain)

Front 14

sickle cell anemia pathogenesis

Back 14

HbS (sickle cell Hb) molecules aggregate and polymerize in hypoxic conditions leading to formation of HbS fibers and distorted red cells, permanent membrane damage from repeated formation of HbS fibers leads to irreversibily sickled cells

Front 15

sickle cell anemia results in...

Back 15

chronic hemolytic anemia and occlusion of small vessels

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sickle cell anemia complications

Back 16

1. fetal hemoglobin inhibits HbS so newborns don't manifest disease for 5-6 months
2. vaso-occlusive crisis or painful crisis represents episodes of hypoxic injury/infarction associated with severe pain in affected region
3. acute chest syndrome results from vaso-occulsion or lung infection
4. sequestration occurs when deformed RBCs fill spleen and cause splenomegaly

Front 17

sickle cell anemia diagnosis

Back 17

HgB elcectrophoresis

Front 18

sicke cell anemia clinical signs

Back 18

autosplenectomy, 2° hemochromatosis, gall stones, leg ulcers

Front 19

thalassemia

Back 19

bone pain?
characterized by a decrease or lack of α or ß-globin chain of HbA α2ß2

Front 20

ß°-thalassemia

Back 20

total absence of ß-globin chains in homozygous state (ß°/ß° or ß*/ß*)

Front 21

ß*-thalassemia

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reduced but detectable ß-globin synthesis in heterozygous state (ß°/ß*)

Front 22

ß-thalassemia pathogenesis

Back 22

reduction in beta chains leads to excess alpha chains in blood, most commonly caused by splicing mutations

Front 23

ß-thalassemia clinical symptoms

Back 23

major: severe transfusion dependent anemia (manifests around 9 months when HbF switches to HbA)
minor: offers resistance against malaria, anisocytosis and target cells

Front 24

hereditary spherocytosis

Back 24

inherited disorder characterized by defect in red cell membrane

Front 25

hereditary spherocytosis pathology

Back 25

RBCs take on spheroidal shape, spectrin, ankyrin, and protein 4.1 are components responsible for normal cell membrane (spectrin deficiency most common)

Front 26

polycythemia

Back 26

increased red cell count (Hb usually increased as well)

Front 27

relative polycythemia

Back 27

results from dehydration (or secondary to a condition causing dehydration)

Front 28

primary absolute polycythemia

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increase in red cell mass resulting from an abnormality of myeloid stem cell

polycythemia vera - neoplastic proliferation of RBCs

Front 29

secondary absolute polycythemia

Back 29

normal red cell progenitors proliferate in response to increased levels of erythropoietin

appropriate: lung disease, high altitude, cyanotic heart disease
inappopriate: erythropoietin secreting tumors, paraneoplastic syndrome

Front 30

bleeding disorder tests

Back 30

1. bleeding time (BT) - time for skin puncture to stop bleeding (2-10 minutes normal)
2. platelet count
3. phrothrombin time (PT) - extrinsic and common coagulation pathways (normal 10-13s), ↑PT results from factor 5, 7, 10, prothrombin, or fibrinogen
4. partial thromboplastin time (PTT) - intrinsic and common clotting pathways (normal 20-34s), ↑PTT results from factor 5, 7, 9, 10, 11, 12, prothrombin, or fibrinogen

Front 31

von willebrand disease

Back 31

most common bleeding disorder, defective von Willebrand factor can no longer provide adhesion between platelets and platelets/subednothelial layer

Front 32

von willebrand disease test results

Back 32

↑BT, ↑PTT, normal plateley levels

Front 33

hemophilia A

Back 33

x-linked recessive, factor 8 deficiency

Front 34

hemophilia A disease test results

Back 34

↑PTT, normal BT, PT, and platelet counts

Front 35

hemophilia B

Back 35

clinically indistinguishable from Hemophilia A, factor 9 deficiency

Front 36

hemophilia B disease test results

Back 36

↑PTT, normal BT, PT, and platelet counts

Front 37

neoplastic proliferation of WBCs (WBC neoplasms)

Back 37

most lymhoid neoplasms are of B-cell origin, all lymphoid neoplasms are derived from a single cell

Front 38

precursor b-cell neoplasms

Back 38

abrupt stormy onset, bone marrow suppression leads to anemia, bleeding, fever, infection, enlarged lymph nodes and spleen

Front 39

chronic lymphocytic leukemia (CLL) and small lymphocytic leukemia (SLL)

Back 39

indistinguishable, most common leukemia of adults, CLL lymphocytes are fragile/rupture during smear (smudge cells), tumor cells express CD19, CD20/T-cell marker only expressed on small subset of B-cells

Front 40

CLL/SLL clinical findings

Back 40

often asymptomatic, easy fatigability, weight loss, anorexia, enlarged lymph spleen

Front 41

follicular lymphoma

Back 41

(14;18) translocation, CD5 not expressed on follicular cells

Front 42

follicular lymphoma clinical findings

Back 42

painless enlarge lymph nodes, incurable

Front 43

diffuse large b-cell lymphoma

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20% of all non-hodgkins lymphoma, treatable, rapidly fatal, negative TdT

Front 44

diffuse large b-cell lymphoma clincal features

Back 44

large destructive masses found in liver or spleen, GI tract/skin/bone/brain may be presenting feature

BCL6 - better prognosis
p53 - worse

Front 45

multiple myeloma

Back 45

bone destruction mediated by IL-6

Front 46

multiple myeloma clinical findings

Back 46

suppression of humoral immunity, hyperviscosity syndrome, hypercalcemia, bence jones protein appears in urine, renal failure

Front 47

Hodgkin's disease

Back 47

three types, arises from single or chain of nodes and spreads, reed sternberg cell is considered to be the neoplastic event

Front 48

nodular sclerosis (hodgkin's disease)

Back 48

most common form of HD, lacunar cell (varient Reed-Sternberg) present

frequent mediastinal involvement, affects females more

Front 49

mixed cellularity (hodgkin's disease)

Back 49

lymph node architecture displays heterogenous cellular infiltrate, RS/mononuclear RS cell seen in lymph nodes, peaks in young adults and adults>55

Front 50

acute myelogenous leukemia (AML)

Back 50

8 subtypes, characterized by accumulation of immature myeloid cells in bone marrow

diagnosed based on finding >20% myeloid blast in bone marrow

auer rods - abnormal azurophilic granules found mostly in M3

Front 51

AML clinical findings

Back 51

gingivival hyperplasia, philadelphia chromosome (9;22) means bad diagnosis

Front 52

chronic myelogenous leukemia (CML)

Back 52

distinguised by translocation of BCR gene on chromosome 9 and ABL gene on chromosome 22, BCR-ABL fusion gene (9;22 philadelphia gene) leads to accelerated cell division

Front 53

CML clinical findings

Back 53

nonspecific initial symptoms, slow progression but patients enter an accelerated phase when untreated, after accelerated phase terminates (9-12 months) symptoms resemble acute leukemia