Sm 1St Aid-Usmle Pathology Heme

Front 1

___% of African-Americans carry the HbS trait, and ___% have the disease.

Back 1

8%; 0.2% (1:500)

Front 2

________ = defect of platelet aggregation

Back 2

Glanzmann's thrombasthenia

Front 3

________ disease = defect of platelet adhesion

Back 3

Bernard-Soulier Disease

Front 4

_____= activation of coagulation cascade leading to microthrombi and global consumption of platelets, fibrin, and coagulation factors.

Back 4

DIC (Disseminated intravascular coagulation)

Front 5

2 therapies for sickle cell anemia:

Back 5

1.Hydroxyurea (increased HbF) 2.BM transplantation

Front 6

50% of Hodgkin's cases are associated with which virus?

Back 6

EBV

Front 7

Alpha-thalassemia is prevalent in which populations?

Back 7

Asia and Africa (A=alpha=asia=africa)

Front 8

Antiplatelet antibodies and increased megakaryocytes are seen in ____.

Back 8

ITP

Front 9

bcl-2 activation is associated with which translocation and which lymphomas?

Back 9

t(14;18) - Follicular lymphomas

Front 10

bcr-abl hybrid is associated with which translocation and which leukemia?

Back 10

t(9;22) - CML

Front 11

Beta-Thalassemia is prevalent in which population?

Back 11

Mediterranean (hint: thalassa=sea. Think, thalaSEAmia)

Front 12

Blood smear of a Multiple Myeloma patient would show what?

Back 12

RBCs stacked like poker chips (Rouleau formation)

Front 13

Burkitt's lymphoma:'______' appearance associated with what virus? Endemic where?

Back 13

'Starry sky'(sheets of lymphocytes w/ interspersed macrophages); EBV; Africa

Front 14

c-myc activation is associated with which translocation and which lymphoma?

Back 14

t(8;14) - Burkitt's

Front 15

Causes of aplastic anemia:

Back 15

Radiation, benzene, chloramphenicol, alkylating agents, antimetabolites, viral agents (Parvovirus B19, EBV, HIV), Faconi's anemia, idiopathic (immune-mediated, primary stem cell defect), acute hepatitis

Front 16

Causes of platelet abnormalities:

Back 16

ITP (antiplatelet antibodies/inc megakaryocytes), TTP (schistocytes), drugs, DIC(inc fibrin split products)

Front 17

CFU-GM (colony forming unit-granulocyte-monocyte) gives rise to which cells?

Back 17

Monocytes, neutrophils and basophils

Front 18

CLL is very similar to what lymphoma?

Back 18

SLL (small lymphocytic lymphoma)

Front 19

Coagulation factor defects (macrohemorrage) cause: (3)

Back 19

1.Hemarthroses (bleeding into joints) 2.easy bruising 3.prolonged PT and/or PTT

Front 20

Common cause of macrocytic megaloblastic anemia:

Back 20

Vit. B12/folate deficiency

Front 21

Compare Multiple Myeloma with Waldenstrom's macroglobinemia:

Back 21

Waldenstrom's also has an M spike, but large amounts of IgM are produced (not IgA or IgG), no lytic lesions

Front 22

Complications often seen in homozygotes (sickle cell disease) include:

Back 22

Aplastic crisis (due to B19 parvovirus infection), autosplenectomy, inc. risk of encapsulated organism infection, Salmonella osteomyelitis, painful crisis (vaso-occlusive), and splenic sequestration crisis (see color image 21)

Front 23

Describe the RBCs of a parient with Hereditary spherocytosis.

Back 23

RBCs are small, round, with no central pallor, less membrane therefore inc MCHC, inc RDW, inc reticulocytes

Front 24

General considerations of leukemias:

Back 24

Inc. number of circulating leukocytes in blood

Front 25

Genetics associated with Burkitt's lymphoma:

Back 25

t(8;14) c-myc gene moves next to heavy chain Ig gene (14)

Front 26

Hb Barts =

Back 26

Gamma-4 tetramers, lacks all 4 alpha globin genes

Front 27

Hb Barts results in what?

Back 27

Hydrops fetalis and intrauterine fetal death

Front 28

HbH =

Back 28

Beta-4 tetramers, lacks 3 alpha globin genes

Front 29

Hereditary spherocytosis causes intrinsic, extravascular hemolysis due to a _____ defect.

Back 29

Spectrin or ankyrin

Front 30

Hereditary spherocytosis is associated with what other problems?

Back 30

gallstones, SPLENOMEGALY, anemia, and jaundice

Front 31

Hereditary spherocytosis is distinguished from warm antibody hemolysis by what test?

Back 31

Direct Coomb's test (Hereditary spherocytosis is Coomb's negative)

Front 32

Hodgkin's or Non-Hodgkin's: which is associated w/ mediastinal lymphadenopathy?

Back 32

Hodgkin's

Front 33

Hodgkin's or Non-Hodgkin's: which is associated with HIV & immunosuppression?

Back 33

Non-Hodgkin's lymphoma

Front 34

Hodgkin's or Non-Hodgkin's: which one displays a bimodal distribution?

Back 34

Hodgkin's (young and old)

Front 35

How does Multiple Myeloma affect calcium levels?

Back 35

Destructive bone lesions cause hypercalcemia (punched-out lytic bone lesions can be seen on x-ray)

Front 36

How is the diagnosis of pancytopenia made?

Back 36

Bone marrow biopsy

Front 37

How many alpha/beta genes are there in hemoglobin?

Back 37

Alpha=4; Beta=2

Front 38

In _____, the alpha globin chain is underproduced.

Back 38

alpha thalassemia (1-4)

Front 39

In _____, the beta chain is underproduced.

Back 39

Beta-minor thalassemia (heterozygote)

Front 40

In ______, the beta chain is absent.

Back 40

Beta-major thalassemia (homozygote)

Front 41

In anemia of chronic disease, are these values increased or decreased? 1.TIBC 2.ferritin 3.serum iron 4.storage iron in marrow macrophages 5.% sat.

Back 41

1.dec. 2. inc. 3.dec. 4.inc. 5.normal

Front 42

In Beta Thalassemia, cardiac failure is often due to what?

Back 42

Secondary hemochromatosis (due to transfusions)

Front 43

In which hemorrhagic disorder is PT increased?

Back 43

DIC

Front 44

In which hemorrhagic disorders is PTT increased? (4)

Back 44

1.Hemophilia A 2.Hemophilia B 3.von Willibrand's disease 4.DIC

Front 45

Increased or decreased in iron deficiency anemia? 1.TIBC 2.ferritin 3.serum Fe

Back 45

1.TIBC=inc 2.serum Fe=dec 3.Ferritin=dec

Front 46

Is Hodgkin's more common in men or women?

Back 46

men (except nodular sclerosing type)

Front 47

Lab findings of DIC:

Back 47

increased PT/PTT, inc. fibrin split products (D-dimers), dec. platelet count

Front 48

Lymphoblastic lymphoma commonly presents with what?

Back 48

ALL and mediastinal mass (LL with ALL)

Front 49

Lymphomas derived from the B cell lineage:

Back 49

ALL (B cell), Lymphoblastic lymphoma, CLL (B), Lymphocytic lymphoma, Follicular center cell lymphoma, Immunoblastic lymphoma (B), Plasmacytoid lymphocytic lymphoma and myeloma

Front 50

Lymphomas derived from the T cell lineage:

Back 50

ALL (T cell) Lymphoblastic lymphoma, CLL (T) Lymphocytic lymphoma, Immunoblastic lymphoma (T), Sezary syndrome and mycosis fungoides

Front 51

Macrocytic anemia is defined as MCV>____.

Back 51

100

Front 52

Marrow failure due to leukemia can lead to (3)

Back 52

1.anemia (dec. RBCs) 2.infections (dec. WBCs) 3.hemorrhage (decreased platelets)

Front 53

Microcytic, hypochromic anemia = MCV<____.

Back 53

80

Front 54

Name 3 coagulopathies (bleeding disorders):

Back 54

1.Hemophilia A 2.Hemophilia B 3.von Willibrand's disease

Front 55

Name 3 etiologies of microcytic, hypochromic anemia:

Back 55

1.iron deficiency 2.Thalassemias 3.lead poisoning

Front 56

Name 5 types of Non-Hodgkin's:

Back 56

1.Small lymphocytic lymphoma 2.Follicular lymphoma (small cleaved cell) 3.Diffuse large cell 4.Lymphoblastic lymphoma 5.Burkitt's lymphoma

Front 57

Name 6 hemorrhagic disorders.

Back 57

1.Qualitative platelet defects 2.Thrombocytopenia 3. Hemophilia A (Factor VIII def.) 4. Hemophilia B (Factor IX def.) 5.Von Willibrand's disease 6.DIC

Front 58

Name 9 chronic myeloid leukemias:

Back 58

CML, Polycythemia Vera, Myelofibrosis, idiopathic thrombocythemia, (chronic monocytic, chronic myelomonocytic, eosinophilic, chronic erythroid) *last 4 are rare*

Front 59

Nodal involvement and spread of Hodgkin's vs. Non-Hodgkin's:

Back 59

Hodgkin's: localized, single group of nodes, extranodal rare, contiguous spread; Non-Hodgkin's: multiple peripheral nodes, extranodal involvement common, noncontiguous spread

Front 60

Normal values: 1.TIBC 2.Serum iron 3.% sat.

Back 60

1.TIBC 250-400 micro grams/dl 2.50-150 3.20-50 %

Front 61

occult blood lose anemia

Back 61

"dec. serum iron, inc. RBC protoporphyrin/transferrin/TIBC.

Front 62

nml ferritin"

Front 63

Other causes of macrocytic anemia (2)

Back 63

1.Drugs that block DNA synthesis (e.g.sulfa drugs, AZT) 2.Marked reticulocytosis

Front 64

pathogenesis of b+ thalassemia

Back 64

promoter region mutation; splicing mutation (most common)

Front 65

pathogenesis of b0-thalassemia

Back 65

chain terminator mutations

Front 66

Pathologic features of aplastic anemia:

Back 66

Pancytopenia with normal cell morphology, hypocellular bone marrow with fatty infiltration

Front 67

Peak incidence of NHL between what ages?

Back 67

20-40

Front 68

Philadelphia chromosome is what?

Back 68

t(9;22), bcr-abl

Front 69

Plasma cells in Multiple Myeloma produce large amounts of what?

Back 69

IgG (55%) or IgA (25%)

Front 70

Platelet abnormalities (microhemorrage) lead to:(4)

Back 70

1.Mucous membrane bleeding 2.Petechiae 3.Purpura 4.Prolonged bleeding time

Front 71

PMNs are hypersegmented in ____ .

Back 71

Vit. B 12 and folate deficiencies

Front 72

PT and PTT: which one measures the intrinsic and which one measures the extrinsic path?

Back 72

PT (extrinsic); PTT (intrinsic)

Front 73

Rank the prognoses of the different types of Hodgkin's:

Back 73

NS and LP = excellent; MC = intermediate; LD = poor

Front 74

Schistocytes are characteristic of ___.

Back 74

TTP and DIC

Front 75

Sickle cell heterozygotes (sickle cell trait) are relatively ____-resistant.

Back 75

Malaria; (balanced polymorphism)

Front 76

Symptoms of aplastic anemia:

Back 76

Fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, infection

Front 77

t(11;14) is associated with which disorder?

Back 77

Mantle cell lymphoma

Front 78

t(11;22) is associated with which disorder?

Back 78

Ewing's sarcoma

Front 79

t(15;17) is associated with which disorder?

Back 79

M3 type of AML

Front 80

The lymphoid stem cell gives rise to: (2)

Back 80

T cells and B cells

Front 81

The majority of Non-Hodgkin's involve T cells or B cells?

Back 81

B cells (except lymphoblastic T cell origin)

Front 82

Treatment of aplastic anemia:

Back 82

Withdrawal of offending agent, allogenic bone marrow transplantation, RBC/platelet transfusion, G-CSF or GM-CSF

Front 83

What are 2 indications of hemolysis?

Back 83

1.decreased serum haptoglobin 2.increased serum LDH

Front 84

What are constitutional signs/symptoms?

Back 84

(mostly seen in Hodgkin's)low grade fever, night sweats, weight loss

Front 85

What are some causes of Normocytic, normochromatic Anemia?

Back 85

Acute hemorrhage, Enzyme defects (G6PD def., PK def.), RBC membrane defects (spherocytosis), Bone marrow disorders (aplasic anemia), Sickle cell, Autoimmune hemolytic anemia, Anemia of Chronic Disease

Front 86

What are some other causes of DIC?

Back 86

gram(-) sepsis, transfusion, trauma, malignancy, acute pancreatitis, and nephrotic syndrome

Front 87

What can cause marrow failure in leukemia?

Back 87

Infiltration of leukemic cells into Bone marrow (BM)

Front 88

What creates a good prognosis for Hodgkin's?

Back 88

Increased lymphocytes and decreased Reed-Sternberg (RS) cells

Front 89

What do you find on blood smear w/ DIC?

Back 89

Helmet-shaped cells and shistocytes

Front 90

What does the skull look like on X-ray for sickle cell anemia?

Back 90

'Crew cut' due to marrow expansion (also seen in thalassemias)

Front 91

What genetics are involved with Follicular lymphoma (small cleaved cell)?

Back 91

t(14;18)bcl-2 expression

Front 92

What is another Beta chain mutation in which patients have a milder disease than HbSS patients?

Back 92

HbC defect; patients can be HbC or HbSC(1 of each mutant gene)

Front 93

What is aplastic anemia?

Back 93

Pancytopenia characterized by severe anemia, neutropenia, and thrombocytopenia caused by destruction of multipotent myeloid stem cells, with inadequate production or release of differentiated cell lines.

Front 94

What is compensatorily increased in both forms of Beta Thalassemia?

Back 94

fetal hemoglobin; however, it is inadequate

Front 95

What is found in the urine of patients with Multiple Myeloma?

Back 95

Ig light chains (Bence Jones protein)

Front 96

What is it called when CML transitions to AML?

Back 96

Blast crisis

Front 97

What is the characteristic cell of Hodgkin's lymphoma?

Back 97

Reed-Sternberg cell (B-cell origin); decreased numbers of RS cells indicates a better prognosis, see image 25

Front 98

What is the M spike?

Back 98

Monoclonal immunoglobin spike on serum electrophoresis

Front 99

What is the MOST common bleeding disorder?

Back 99

von Willibrand's disease (deficiency of von Willibrand's antigen)

Front 100

What is the MOST common cause of DIC?

Back 100

obstetric complications

Front 101

What is the MOST common primary tumor arising within bone in adults?

Back 101

Multiple Myeloma

Front 102

What is the shape of RBCs in sickle cell anemia?

Back 102

Crescent shape

Front 103

What Leukemia type has AUER rods, myeloblasts, and occurs in adults?

Back 103

AML (myeloblasts)

Front 104

What Leukemia type is common in children, has lymphoblasts, is MOST responsive to therapy, and may spread to CNS/testes?

Back 104

ALL (pre-B or pre-T cells)

Front 105

What Leukemia type is MOST commonly associated with Philadelphia chromosome (9:22 translocation->bcr-abl), myeloid stem cell proliferation, and may accelerate to AML?

Back 105

CML (Myeloid stem cells)

Front 106

What Leukemia type occurs in older adults, has lymphadenopathy, hepatosplenomegaly, few symptoms, indolent course, inc. smudge cells in peripheral blood smear, warm Ab autoimmune hemolytic anemia?

Back 106

CLL (lymphocytes Non-antibody-producing B cells)

Front 107

What mutation causes sickle cell anemia?

Back 107

HbS mutation is a single aa replacement in the Beta chain (substitute normal glutamic acid with valine)

Front 108

What other problems result from Multiple Myeloma?

Back 108

Renal insufficiency, increased susceptibility to infections, anemia, and amyloidosis

Front 109

What precipitates sickling of cells?

Back 109

Low oxygen or dehydration

Front 110

What test is used to confirm Hereditary spherocytosis?

Back 110

Osmotic fragility test

Front 111

What test is used to distinguish between immune vs. non-immune RBC hemolysis?

Back 111

Direct Coomb's test

Front 112

What type of cell is cancerous in Multiple Myeloma and what does it resemble?

Back 112

Monoclonal plasma cell, 'fried egg' appearance

Front 113

Where can leukemic cell infiltrate?

Back 113

Liver, spleen, and lymph nodes

Front 114

Which 2 of 6 hemorrhagic disorders have decreased platelet counts?

Back 114

1.thrombocytopenia 2.DIC

Front 115

Which 2 types of Non-Hodgkin's occur in children?

Back 115

Lymphoblastic lymphoma, and Burkitt's lymphoma, 20% of diffuse large cell type too

Front 116

Which Beta Thalassemia results in severe anemia?

Back 116

Beta Thal. major (requires blood transfusions)

Front 117

Which coagulation factors are a part of the intrinsic and which are a part of the extrinsic path?

Back 117

PT(extrinsic):Factors II,V,VII,X; PTT(intrinsic):all factors except VII/XIII REMEMBER THE FACTORS!

Front 118

Which coagulation factors are deficient in each of the Hemophilias?

Back 118

Hemophilia A (factor VIII deficiency); Hemophilia B (factor IX deficiency)

Front 119

Which one is associated with neurological problems, folate deficiency or Vit. B12 deficiency?

Back 119

Vit. B12

Front 120

Which type of Hodgkin's accounts for 6% of cases and which type is the most rare/prognosis.

Back 120

Lymphocyte Predominant (LP) - 6%; Excellent prognosis

Front 121

Which type of Hodgkin's commonly affects males under 35?

Back 121

Lymphocyte Predominant (LP)

Front 122

Which type of Hodgkin's commonly affects older males with disseminated disease?

Back 122

Lymphocyte Depleted (LD)

Front 123

Which type of Hodgkin's has the most Reed Sternberg cells?

Back 123

Mixed Cellularity

Front 124

Which type of Hodgkin's is characterized by collagen banding/lacunar cells?

Back 124

NS (nodular sclerosing)

Front 125

Which type of Hodgkin's is rare?

Back 125

Lymphocyte Depleted (LD) - rare; Poor prognosis

Front 126

Which type of Hodgkin's is the 2nd most common/prognosis? (25%)

Back 126

Mixed cellularity (MC); Intermediate prognosis

Front 127

Which type of Hodgkin's is the MOST common/prognosis? (65-75%)

Back 127

NS (nodular sclerosing); Excellent prognosis

Front 128

Which type of Hodgkin's primarily affects young adults, women>men?

Back 128

Nodular Sclerosing (NS)

Front 129

Which type of Non-Hodgkin's clinically presents like CLL?

Back 129

Small lymphocytic lymphoma (SLL with CLL)

Front 130

Which type of Non-Hodgkin's is difficult to cure?

Back 130

Follicular lymphoma

Front 131

Which type of Non-Hodgkin's is MOST common in children?

Back 131

Lymphoblastic lymphoma (very aggressive)

Front 132

Which type of Non-Hodgkin's is the MOST common type in adults?

Back 132

Follicular lymphoma (small cleaved cell)

Front 133

Which types of Non-Hodgkin's involve T cells?

Back 133

Lymphoblastic lymphoma (immature T cells); 20% of Diffuse large cell Non-Hodgkin's (mature T cells)

Front 134

With iron overload (hemosiderosis) are values increased or decreased? 1.TIBC 2.serum iron 3.%sat.

Back 134

1.TIBC normal 2.Serum iron increased 3.% sat. increased (100%)