Renal Diseases Of Blood

Front 1

How many WBCs should there be in a liter of blood?

Back 1

WBCs: in health, there should be 4.4-11.3 x 109/L of WBCs in blood.

Front 2

What percent of plasma cells are found in blood?

Back 2

• Its important to know that in health, there should never be plasma cells in the blood.

Front 3

Which leukocyte includes the following properties?
Bactericidal
“left shift”: increase in immature precursors

Back 3

Neutrophils (PMNs)

Front 4

Which leukocyte includes the following properties?
allergic, anti-parasitic, drug, neoplasms

Back 4

Eosinophils

Front 5

Which leukocyte includes the following properties?
immediate hypersensitivity reactions; ie, asthma

Back 5

Basophils

Front 6

Which leukocyte includes the following properties?
“the mononuclear phagocyte system;”
blood monocytes + those carried to peripheral tissues-> histeocyte or macrophage:

bacteria, fungi, mycobacteria, protozoa, virus, take up senescent cells, antigen presentation

Back 6

Monocytes

Front 7

What is the percentage of T lymphocytes compared to the rest of the lymphocytes?

Back 7

80-90% of Lymphocytes

Front 8

What is leukopenia usually attributed to?

Back 8

A decrease in neutrophils (i.e. neutropenia or granulocytopenia)

Front 9

What are the causes of Lymphopenia?

Back 9

HIV
Glucocorticoids
Drugs
Autoimmune
Acute viral
Malnutrition

Front 10

What can be due to Decreased Production & Increased Destruction?

Back 10

Neutropenia and Agranulocytosis

Front 11

What are some Drugs that are attributed to increased destruction in Neutropenia and Agranulocytosis?

Back 11

◊ Alkylating agents, antimetabolites

Choramphenical, sufonamides, chlorpromazine, thiouracil

Front 12

What are the clinical manifestations of Neutropenia & Agranulocytosis?

Back 12

Infections (deep w/gray, green-black necrotic membranes)
Ulcerating Lesions of the:
*Gingiva
*Floor of Mouth
*Buccal Mucosa
*Pharynx
*Oral Cavity (agranulocytic angina)
*Elsewhere in body

Front 13

What are the 5 pools?

Back 13

1. Precursors
2. Storage
3. Circulating
4. Marginating (cells stuck to the walls of the vessels)
5. Tissues

Front 14

What condition occurs due to too many leukocytes?

Back 14

Reactive Leukocytosis

Front 15

What is the difference in site and spread between Hodgkin's Lymphoma & NHL?

Back 15

Hodgkin - Single node; contiguous; Rarely involves waldeyer ring & mesentery

NHL - Multiple nodes; Extranodal; Unpredictable spread; Waldeyer ring & mesentery

Front 16

What are the broad classifications for Neoplastic Lymphoid Disorders?

Back 16

1. Lymphoid Neoplasms (has Hodgkin's Lymphoma)
2. Myeloid Neoplasms
3. Histiocytoses

Front 17

What is the characteristic cell found in Hodgkin's Lymphoma?

Back 17

Reed-Sternberg

Front 18

What are these classified as?
*Acute myelogenous leukemias
*Myelodysplastic syndromes
*Chronic myeloproliferative disorders

Back 18

Myeloid Neoplasms

Front 19

What cell has the following properties?

in a non-neoplastic inflammatory background (EOS)
Classically has two big nuclei that look like owl eyes.

Back 19

Reed-Sternberg

Front 20

What kind of cells does non-classical hodgkin lymphoma have?

Back 20

Popcorn or L&H cells (lymphocytic & histeocyte Reed-Sternberg variants)

Front 21

What are the types of classical Hodgkin's Lymphoma?

Back 21

1. Nodular Sclerosis
2. Mixed Cellularity
3. Lymphocyte Rich
4. Lymphocyte-depleted (least favorable diagnosis)

Front 22

What is the most common type of lymphoma?

Back 22

Chronic Lymphocytic Leukemia (CLL)/Small Lymphocytic Lymphoma (SLL)

Front 23

What type of lymphoma Always involves bone marrow & commonly the spleen. It also has Cytopenias: bleeding & infection?

Back 23

CLL/SLL

Front 24

What is the most common NHL?

Back 24

Follicular Lymphoma

Front 25

What type of lymphoma has t(14:18)?

Back 25

Follicular Lymphoma

t(14:18) 14 is the heavy change locus of immunoglobulins

Front 26

What type of lymphoma is: *Indulent & Incurable
*BCL-2 expression (normal germinal centers are BCL-2 neg)
*t(14:18)
*A lymph node w/many follicles; normal architecture effaced

Back 26

Follicular Lymphoma

Front 27

What type of lymphoma has t(11:14)?

Back 27

Mantle Cell Lymphoma

Front 28

What type of lymphoma has the properties:
*t(11:14)
*Cyclins D-1 protein

Back 28

Mantle Cell Lymphoma

Front 29

What type of lymphoma ARISES AS A SINGLE RAPIDLY GROWING, SYMPTOMATIC MASS?

Back 29

Diffuse Large B-cell Lymphoma

Aggressive- requires intense chemotherapy
Large neoplastic cell size, diffuse growth pattern

Front 30

What are the different types of plasma cell neoplasms?

Back 30

MGUS (monoclonal gammopathy of uncertain significance)
Waldenstrom Macroglobulinemia

Front 31

What is the prognosis of multiple myeloma?

Back 31

Poor Prognosis
Die within 3-5 years

Front 32

What is the plasma cell content in bone marrow for Multiple Myeloma?

Back 32

>30% plasma cells in marrow

Front 33

What type of lymphoma displays symptoms of Infection, Renal Insufficiency, Hypercalcemia (due to the break-down of bone)

Back 33

Multiple Myeloma

Front 34

What is significant regarding ALLs & AMLs?

Back 34

They can have identical signs and symptoms

Front 35

Who is affected most in ALL (Acute Lymphoblastic Leukemia)?

Back 35

Younger people are affected
(80% B-cell precursors, 20% T-cell precursors)

Front 36

Who is affected most in AML (Acute Myelogenous Leukemia)?

Back 36

Adults

Front 37

What does the cellular composition of AML look like?

Back 37

Have looser nuclei
Prominent nucleoli
More cytoplasm often with Granules

Front 38

What type of lesions are common with AML (Acute Myelogenous Leukemia)

Back 38

Mouth/Throat Ulcers

Front 39

**What type of leukemia has the following properties?
*t(15:17) PML/RARα
*usually present w/DIC
*Response to RETINOIC ACID as a treatment

Back 39

Acute Promyelocytic Leukemia

Front 40

What has Birbeck granules in the cytoplasm (electron microscopy)?

Back 40

Langerhans Cell Histiocytosis

(neoplastic cell is a dendritic cell)

Front 41

What are the different types of Myeloproliferative Disorders?

Back 41

Myeloproliferative Disorders

Chronic Myelogenous Leukemia (CML)
Polycythemia Vera
Essential Thrombocytosis
Primary Myelofibrosis

Front 42

What are common symptoms of Myeloproliferative Disorders?

Back 42

Splenomegaly
Spent phase cytopenias and marrow fibrosis
Can progress over time to acute leukemia (CML invariably does)

Front 43

What type of Myeloproliferative disorder has a Philadelphia (Ph1) chromosome: t(9:22)?

Back 43

Chronic Myelogenous Leukemia (CML)

Translocation is present in 95% of cases
BCT-ABL fusion (tyrosine kinase activity; unregulated myeloproliferation!)
Gleevec (imtinib) inhibits tyrosine kinase

Front 44

What stage of Acute Leukemia (AML) is M0?

Back 44

minimally differentiated

Front 45

What stage of Acute Leukemia (AML) is M1?

Back 45

Without maturation

Front 46

What stage of Acute Leukemia (AML) is M2?

Back 46

With maturation (AUER RODS)

Front 47

What stage of Acute Leukemia (AML) is M4?

Back 47

Myelomonocytic

Front 48

What stage of Acute Leukemia (AML) is M5?

Back 48

M5a = Monoblasts
M5b = Monocytic

Front 49

What stage of Acute Leukemia (AML) is M6?

Back 49

Erythroleukemia

Front 50

What stage of Acute Leukemia (AML) is M7?

Back 50

Magakaryoblastic

Front 51

What condition has the following features?
• Men,50-60 yrs.
• Multiple skeletal sites “punched out” lesions
§ Plasma cells invade and erode bone.
• Erode bone
• pathologic fractures
• Marrow: for diagnosis, you will have to have >30% plasma cells
§ You should normally have no more than 2%
• M-component: 55% IgG;
§ serum IgG>3.5g/dL,IgA>2g/dL
• *Rare to see plasma cells in peripheral blood
• *Infection, renal insufficiency, hypercalcemia (due to the breakdown of bone)
• Poor prognosis: Die within 3-5 years

Back 51

Multiple Myeloma

Front 52

What disorder has a Monoclonal spike of IgG?

Back 52

Multiple Myeloma

Front 53

Leukemia Clue: Children

Back 53

ALL

Front 54

Leukemia Clue: Myeloblasts

Back 54

AML

Front 55

Leukemia Clue: Auer Rods

Back 55

AML, promyelocytic

Front 56

Leukemia Clue: DIC

Back 56

Promyelocytic

Front 57

Leukemia Clue: Elderly

Back 57

CLL
(chronic lymphocytic leukemia)

Front 58

Leukemia Clue: Splenomegaly

Back 58

CML

Front 59

Leukemia Clue: Philadelphia Chromosome

Back 59

CML

Front 60

Leukemia Clue: Tartrate-resistant acid phosphatase

Back 60

Hairy Cell

Front 61

Leukemia Clue: HTLV-1

Back 61

Adult T cell

Front 62

What is the major difference between Multiple Myeloma & Waldenstrom Macroglobulinemia?

Back 62

Lack of lytic bone lesions in Waldenstrom

Front 63

What is the major difference between Acute Leukemias and Chronic Leukemias?

Back 63

Acute Leukemias have blasts in peripheral blood and decreased mature cells

Chronic Leukemias have an increased number of mature WBCs in the peripheral blood

Front 64

What is significant about Leukocyte alkaline phosphatase?

Back 64

It is elevated in inflammatory leukocytosis

It is depressed in chronic myelogenous leukemia

Front 65

Where is factor VIII synthesized?

Back 65

Factor VIII is synthesized in the endothelium of vessels

(the other clotting factors are synthesized in the liver)

Front 66

What condition has a JAK-2 mutation (pH absent) & Low erythropoietin levels (vs. relative polycythemias)

Back 66

Polycythemia Vera

Front 67

What type of lymphoma is associated w/EBV and displays a starry sky pattern?

Back 67

Burkitt Undifferentiated Lymphoma

Front 68

What are normal levels of hemoglobin (gm/dL)?

Back 68

Male 13.6-17.2
Female 12-15

Front 69

What are normal levels of HCT (hematocrit)?

Back 69

Male 39-49
Female 33-43

Front 70

What is the normal MCV (mean cell volume)?

Back 70

82-96

Front 71

What is the definition of anemia?

Back 71

Reduced Oxygen carrying capacity of blood

Front 72

What lab values are reduced in anemia?

Back 72

hematocrit HCT
hemoglobin Hb

Front 73

_______ is the percentage of whole blood that is RBCs

Back 73

Hematocrit

Front 74

___________________ is the coefficient of variation of red blood cell volume

Back 74

Red Blood Cell Distribution Width (RDW)

Front 75

_____________ is the average concentration of Hb in a volume of RBCs

Back 75

Mean cell hemoglobin concentration (MCHC)

Front 76

___________ is the average content of Hb per RBC

Back 76

Mean Cell Hemoglobin (MCH)

Front 77

_________ is the average volume of a RBC

Back 77

Mean Cell Volume (MCV)

Front 78

What are the causes of Megaloblastic anemia?

Back 78

B12, Folate deficiency
Drug use

Front 79

Why do RBC's need to have highly pliable membranes?

Back 79

To navigate between splenic sinusoids

Front 80

What type of anemia has a membrane that is less stable and inflexible resulting in the erythrocyte not being able to leave the spleen. Condition is congenital and the RBC life span is decreased to 10-20 days?

Back 80

Hereditary Spherocytosis (hemolytic anemia)

**Mutation in ANKYRIN, a component of the RBC membrane

Front 81

What type of anemia protects against malaria?

Back 81

Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD)

Front 82

What shows up clinically as hemolysis after exposure to oxidant stress (drugs, foods-fava beans, infection)?

Back 82

G6PD deficiency

Front 83

What is shown in this picture and what anemia causes it?

Back 83

Bite cells & Heinz Bodies

Caused by G6PD deficiency

Front 84

In sickle cell disease defective hemoglobin is produced due to ________

Back 84

Valine instead of Glutamic acid

Front 85

Among those with sickle cell disease (S/S), what percentage have what types of hemoglobin?

Back 85

80% HbS
20% HbF
NO HbA

Front 86

Among those with sickle cell trait(A/S), what percentage have what types of hemoglobin?

Back 86

60% HbA
40% HbS

Front 87

What is the abnormal hemoglobin in sickle cell patients?

Back 87

HbS

Front 88

What is the % hemoglobin of normal adults?

Back 88

96% HbA

Front 89

What is the % hemoglobin of normal babies?

Back 89

75% HbF
25% HbA

Front 90

What is the problem with HbS being sticky?

Back 90

The stickiness leads to cells “hanging” around in these sluggish areas; which exposes them to hypoxic environmnet, the sickling persists, the tissues become inflamed, blood flows EVEN SLOWER, WBCs release pro-imflammatory cytokines; it’s a vicious cycle!!!

Front 91

What are the treatments for sickle cell anemia?

Back 91

Hyroxyurea: increases HbF, which inhibits polymerization of HbS, decreased inflammation

Crises: exchange transfusion

Front 92

What are Thalassemias?

Back 92

Decreased synthesis of NORMAL alpha or beta hemoglobin chain

Front 93

What is the problem we run into with Alpha Thalassemia?

Back 93

Excess of unpaired Beta chains

Front 94

What is the problem we run into with Beta Thalassemia?

Back 94

Excess of unpaired Alpha chains

Front 95

Where does hemolytic anemia (increased destruction)occur?

Back 95

Mostly in Spleen (EXTRAVASCULAR HEMOLYSIS)

Rarely in the vessels (INTRAVASCULAR HEMOLYSIS)

Front 96

What type of anemia is congenital with a mutation in ANKYRIN, a component of the RBC membrane?

Back 96

Hemolytic Anemia: HS (hereditary spherocytosis)

Front 97

What type of anemia protects against Malaria (plasmodium falciparum)?

Back 97

Hemolytic Anemia: G6PD deficiency

Glucose-6-Phosphate Dehydrogenase Deficiency

Front 98

What is:
- An intermittent hemolytic anemia of OLDER RBCs
- Hemolysis after exposure to oxidant stress (drugs, foods-fava beans, infection)
- Heinz bodies (MACROPHAGES IN SPLEEN PLUCK THEM OUT OF MEMBRANE -> BITE CELLS

Back 98

Hemolytic Anemia: G6PD Deficiency

Front 99

In Hemolytic Anemia: Sickle Cell Disease, those with disease have what type of hemoglobin?

Back 99

80% HbS
20% HbF

**NO HbA**

Front 100

In Hemolytic Anemia: Sickle Cell Disease those with trait (A/S) what is the hemoglobin content?

Back 100

60% HbA
40% HbS

Patient functions fairly well

Front 101

Which Thalassemia targets cells?

Back 101

Beta Thalassemia

Front 102

What condition has an acquired defect in RBC membrane, a mutation in phosphatidykinositol glycan A (PIGA), a GPI protein

Back 102

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Front 103

Which disease has RBCs susceptible to lysis by complement - there is a decrease in ALL GPI proteins

*CD55, 59

Back 103

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Front 104

In Paroxysmal Nocturnal Hemoglobinuria (PNH), ALL RBCs ARE NOT AFFECTED. Why?

Back 104

A mutant clone exists as well as normal RBCs Present

Front 105

In RBC destruction due to trauma what shows up on a peripheral smear?

Back 105

RBC fragments AKA SCHISTIOCYTES

Front 106

What test for Immunohemolytic anemia demonstrates AB or complement bound to patient's RBCs?

Back 106

DAT (direct antiglobulin test) "Coombs Test"

- antihuman Ig
- a + test has clumping/agglutination

Front 107

What disease has laboratory findings of Hypochromic microcytic anemia (Decreased MHC & MCV)?

Back 107

Iron Deficiency Anemia

Front 108

What is important regarding anemia of chronic disease?

Back 108

Impaired Fe utilization secondary to a chronic disease (i.e. rheumatoid arthritis, infections)

Front 109

What type of anemia has Hypersegmented neutrophils & pancytopenia?

Back 109

megaloblastic anemia

Front 110

What are the properties of polycythemia?

Back 110

- Non-clonal increase in the concentration of red blood cells unlike myeloproliferative disorders
- Causes: Increased EPO (tumor or hypoxemia) & Dehydration
- Clinically patients present w/difficulty breathing & thrombosis
- Treatment includes therapeutic phlebotomy or hydration

Front 111

What type of anemia presents clinically as:
- mucocutaneous bleeding and infections
- splenomagaly
- reticulocytopenia
- normocytic, normochromic anemia

Back 111

Aplastic Anemia
***************

Front 112

Will vessel wall damage affect blood test results?

Back 112

No