Heme Textbook questions chapter 10-17 + Questions in class

Which of the following is not characteristic of hemoglobinopathies?

All are manifested in clinically significant conditions

which of the following are used in the nomenclature system for abnormal hemoglobin's?

ALL the above

what is the amino acid substitution found in sickle cell anemia?

substitution of valine for glutamic acid at the 6th position from the N-terminal beta chain

what factors contribute to the sickling of RBC's?

decrease in pH, oxygenation, and dehydration

which of the following crises are associated with sickle cell anemia?

ALL the above

what are the therapeutic goals in the treatment of sickle cell anemia?

ALL the above

what is the amino acid substitution found in HDC disease?

substitution of lysine for glutamic acid in the sixth position from the N-terminal beta chain

Which of the following is not true for HBC disease?

HBC can be separated from other hemoglobin's at alkaline pH

Which finding would be most useful in establishing a diagnosis for HbSC disease?

hemoglobin electrophoresis at alkaline pH

Which of the following is a cause of methemoglobinemia?

ALL of the above

What is the hemoglobin defect found in thalassemia syndromes?

Defective production of the globin portion

What type of globin chains and hemoglobin are characteristic of severe alpha thalassemia

four beta (HbH) or four gamma chains (Hb Bart's )

The enzymatic contents of primary azurophilic granules are:

Meyloperoxidase and lysosome

Directional migration towards a gradient stimulated by a chemoattractant is referred to as:

Chemotaxis

The marking of an invading microbe with IgG and complement to facilitate recognition is refered to as:

opsonization

which sequence reflects the correct sequence of phagocytosis?

binding, ingestion, fusion, release of cytoplasmic granules

In O2-dependent killing, the enzyme responsible for mediating the production of of active O2 metabolites the respiratory burst is:

NADPH oxidase

the 2 most important biochemical products of the respiratory burst that are involved with particle digestion during active phagocytosis are:

superoxide anion and hydrogen peroxidase

The main morphological characteristics associated with Chidek Higashi syndrome are:

giant lysosomal granules

The defect in chronic granulomatous disease is attributed to:

defective bacterial killing

Identify disease characteristics of Chidek-Higashi syndrome:

partial albinism, recurrent infections, and mild bleeding tendencies

Pelger Huet anomaly may be described by:

Hyposegmentation of nucleus with majority of neutrophils being bilobed or mono-lobed

reactive lymphocytes may be distinguished from blasts by the presence of which of the following morphological features?

heterogenous cell populations

Which of the following antigens is detectable first by ELISA?

EBV-VCA (IgM)

the Epstein-Barr virus infects which type of cell?

B lymphocytes

What is the most frequent cause of heterophile (Monospot) negative mononucleosis-like syndrome?

CMV

in which of the following conditons are reactive lymphs found?

ALL of the above

Which of the following lab findings would be most characteristic of anemia of chronic idease?

normocytic normochromic anemia with low SI levels, low TIBC, and high ferritin

which of the following are causes of anemia of chronic disease?

ALL of the above

What are the main factors responsible for reducing erythropoies in anemia of chronic disease?

cytokines from macrophages and lymphocytes

what is the treatment for anemia of chronic disease?

treatment of the underlying inflammatory process

Which of the following red cell membrane deficiencies do not cause hereditary spherocytosis?

protein 4.1

which of the following lab tests would not be typical of hereditary spherocytosis?

low mchc

Which of the following abnormalities is/are thought to cause severe fragmentation and micro-spherocytes characteristic of hereditary pokilocytosis?

defective membrane spectrin tetramer assembly and spectrin deficiency

which of the following are not typical of south-east asian ovalocytosis?

deficiency of band 3

which of the following are classified as disorders of membrane cation permeability?

hereditary stromacytosis and hereditary xerocytosis

Which of the following is not true for HbC disease?

HbC can be separated from other hemoglobin's at alkaline pH during electrophoresis

Which finding would be most useful in establishing a diagnosis for HbSC disease?

Hb electrophoresis at alkaline pH

Which of the following are causes of methemoglobinemia?

ALL of the above

what are mechanisms for immune hemolysis?

ALL of the above

Which would best distinguish hemolytic anemia caused by immune mechanisms from other hemolytic anemias?

Positive DAT

what is true concerning autoimmune hemolytic anemia?

antibodies are produced against one's own erythrocyte antigens

What is the process by which the immune system produces antibodies against foreign red blood cells introduced into their circulation via transfusion or pregnancy?

alloimmune hemolytic anemia

what causes HDFN?

Maternal IgG antibodies form from previous pregnancy or blood exposure, cross the placenta, and attach to fetal cells

Which of the following is not characteristic of warm autoimmune hemolytic anemia?

positive result for the donath-landsteiner test

what are features of the cold agglutin system?

ALL OF THE ABOVE

What are the principles of the Donath-Landsteiner test?

antibody binds red cells at 4C and causes lysis at 37C

What are the causes of nonimmune hemolytic anemia?

ALL of the above

which of the following organism are associated with hemolytic anemia

ALL the above

Which of the following is measured by an automated hematologic instrument that would be most likely effected by cold agglutinin?

hematocrit

What is the most common glycolytic enzyme deficiency associated with the pentose phosphate pathway?

G6PD deficiency

What is the most common glycolytic enzyme deficiency associated with the Embden-Myerhof pathway?

Pk deficiency

Oxidative denaturation of hemoglobin results in the formation of small particles that are visualized by a super-vital stain. What are the particles?

Heinz bodies

In the evaluation of a patient with G6PD deficiency, which of the following test results would most likely indicate a deficiency of this enzyme?

ALL of the above

what lab test result would indicate that a patient is

Pk deficent?

abnormal rate of hemolysis that is independent of the presence or absence of glucose in incubation

What deficiency causes hemoglobin to be oxidized from the ferrous to ferric state?

NADH-methemoglobin reductase deficiency

what happens when normal donor red cells are transfused into a patient with an intracorpuscular red cell defect?

donor cells have normal survival

Which of the following tests are not used to detect increased red cell destruction?

reticulocyte count

a anemic patient is investigated for a hemolytic state. they have the following results: Hgb=8g/dl, Hct=23%, retic count=8%, polymorphs on the peripheral smear, what is the RPI?

2

what tests are useful in identifying the cause of red cell hemolysis?

coombs test

Which of the following is NOT characteristic of hemoglobinopathies?

All are manifested in clinically significant conditions

which of the following are used in nomenclature of abnormal hemoglobin-pathies?

ALL of the above

what is the AA substitution found in sickle cell anemia?

substitution of valine for glutamic acid at the 6th position of the beta globin chain

what factors contribute to the sickling of red blood cells?

decrease in pH, oxygenation, and dehydration

Which of the following are crises associated with sickle cell anemia?

ALL of the above

what are the therapeutic goals in treating sickle cell?

All of the above

what is the amino acid substitution found in HbC disease?

substitution of lysine for glutamic acid at the 6th position of the beta globin chain

-blasts are tdt negative

-varying amount of SBB positive

-50% stain positive for Nonspecific esterase

what type of leukemia is this?

Acute myelomonocytic leukemia

-high wbc count, low platelets

- Netative for peroxidase and NSE

- tdt positive

- HLA-DR+, CD-19+, CD-10+, Cmu-, sIg-, CD-7-

what is the most likely diagnosis?

Precursor B-ALL with t(12;21) TEL-AML1

-high wbc count, anemia and thrombocytopenia

- 80% blasts, 15% neutrophils and 5% lymphocytes

- MPO positive, NSE negative

- CD13, CD33, CD34, CD117 positive

what is the diagnosis?

AML without maturation, FAD M1 type

- 90% blasts, 6% segs, 4% monos

- blasts are large with relatively abundant cytoplasm

- more than 90% of them are positive for NSE

- occasional blast is SBB positive

What is the most likely diagnosis?

AML, Acute monoblastic leukemia, FAB M5 type

-hypercellular marrow

-60% blasts

- tdt positive

- CD3, 10, 7, 13, 34, 45 all positive

-no surface Ig's

what is the most likely diagnosis?

ALL, precursor T cell type

- DIC

- low WBC count

- 95% atypical mononuclear cells

- packed with large purple-staining granules

- multiple Auer rods

- Peroxidase positive

What is the most likely diagnosis?

AML, FAB M3

- medium-sized blasts in BM smear

- numerous liquid vacuoles

- t(8;14) involving MYC on Chromosome 8 and Ig HC on chromosome 14

Flow cytometry will show what?

CD10, 19,20,45 positive and a surface kappa LC

t(1;19) PBX-E2A is a associated with a ________________ prognosis

unfavorable

what cytochemical stain best helps differentiate ALL from AML?

MPO

how many blast cells are necessary for acute leukemia to be classified under FAB?

30%

how many blast cells are necessary for acute leukemia to be classified under WHO?

20%

what chromosome abnormality occurs in the FAB M2 type of AML?

t(8;21)

What cytochemical stain is good for separating myeloblasts from monoblasts?

Nonspecific esterase (NSE)

what chromosome abnormality occurs in FAB M3 abnormaility?

t(15;17)

What is the most common glycolytic enzyme deficiency associated w the pentose phosphate pathway (aerobic pathway)?

G6PD deficiency

What is the most common glycolytic enzyme deficiency associated with the Embden-Meyerhof pathway (anaerobic pathway)?

PK deficiency

Oxidative denaturation of hgb results in the formation of small particles that are visualized with supravital staining referred to as:

Heinz bodies

In the evaluation of a patient for G6PD deficiency, which of the following test results would indicate a deficiency of the enzyme?

Increase fluorescence in the fluorescent spot test

Which of the following represents the second most frequently encountered enzyme deficiency that cause hemolytic anemia?

PK

Which of the following types of G6PD enzyme is the most common form in all populations and exists in 99% of whites in the US?

GD B

Due to the need to remove the spleen in PK, which of the follow inc significantly in PTs?

Reticulocytes

Due to the frequency of transfustions w/ PK deficiency, what do patients often experience as a result

Iron overload

Which of the following is true about acquired methemoglobinemia?

Often an acute, but manageable condition

What % of Hgb variations represents single AA substitutions in the globin chains?

90

Quantitative hemoglobinopathies characterized by a decreased production of Hb with A decreased synthesis of one globin chain

thalassemias

Which of the follow doesnot represent a major category of hemoglobinopathies

Hemoglobin with abnormal oxygen function

Which of the following does not represent embryonic hgb formed only in the embryonic stages of human development?

Alpha2delta2

In contrast to beta thal, alpha thal usually manifests:

Immediately at birth

Which of the following findings would not be indicative of heterozygous B thal?

Hgb A level of 65 - 85%

Which form of HPFH is characterized by uniform distribution among red cells when stained by KB methods?

Pancellular

Complement is a group of serum proteins that interact with each other to bring about

Complement-dependent cell lysis

Activation of classical pathway involves with types of Ig's?

IgM and IgG

The mechanism of direct lysis resulting from the release of lytic enzymes from effector cells that act on the red cell membrane is called?

Antibody-dependent cellular cytotoxicity