HEME final

According to the WHO classification of MDS, what percentage of blasts would constitute transformation to an acute leukemia?

20%

Through routine screening, prospective parents discover that they are both heterozygous for Hb S. what percentage of their children potentially could have sickle cell anemia (HbSS)?

25%

Into what other hematologic disease does MDS often convert?

AML

Acanthocytes are found in association with:

Abetalioproteinemia

A 5 year old girl was seen by her physician several days prior to this visit and was diagnosed with pneumonia. Her mother has brought her to the physician again because the girl's urine began to darken after the first visit and now is alarmingly dark. The girl has no history of anemia, and there is no family history of any hematologic disorder. The CBC shows a mild anemia, polychromasia, and a few schistocytes. This anemia could be categorized as:

Acquired, fragmnetation

The most common manifestation of G6PD deficiency is:

Acute hemolytic anemia caused by drug-induced exposure or infections

Immune hemolytic anemia is due to a(n):

Allo- or autoantibody against an RBC antigen

The altered shape of the spherocyte in HS is due to:

An abnormal RBC membrane protein affecting vertical protein interactions

Physiologic programmed cell death is termed:

Apoptosis

The screening test for Hb S that uses a reducing agent, such as sodium dithione, is based on the fact that hemoglobin that sickle:

Are insoluble in reduced, deoxygenated form

Chronic secondary CAD is most often associated with

B cell malignancies

1One week after returning from a vacation in rhode island, a 60 year old man experienced fever, chills, nausea, muscle aches, and fatigue of 2 days' duration. A CBC showed a WBC coutn of 4.5x10^9/L, hemoglobin level of 10.5 g/dL, a platelet count of 134x10^9/L, and a reticulocyte count of 2.7%. The medical laboratory scientist noticed tiny ameboid ring forms in some of the RBCs and some tetrad forms in others. These findings suggest:

Babesiosis

Which of the following is an erythrocyte progenitor?

CFU-E

A peripheral blood film that shows increased neutrophils, basophils, eosinophils, and platelets is highly suggestive of:

CML

The glycolipids of the RBC membrane:

Carry RBC antigens

Which of the following is not a mechanism causing anemia in P. falciparum infections?

Competition for vitamin B12 int he erythrocyte

The pathophysiology of immune hemolysis with IgM antibodies always involves:

Complement

Which one of the following morphologic changes occurs during normal blood cell maturation

Condensation of nuclear chromatin

Which anemia should be suspected in a patient with a fractory anemia, reticulocytopenia, hemosiderosis, and binucleated erythrocyte precursors in the bone marrow?

Congenital dyserythropoietic anemia

Which of the following helps maintain RBC shape?

Cytoskeleton proteins

Which of the following laboratory results may be seen in BOTH traumatic cardiac hemolytic anemia and exercise- induced hemoglobinuria?

Decreased serum haptoglobin

The RBCs in HE are abnormally shaped and have unstable cell membranes as a result of:

Defects in horizontal membrane protein interactions

The most important finding in the diagnostic investigation of a suspected autoimmune hemolytic anemia is:

Demonstration of IgG and/or C3d onthe RBC surface

A qualitative abnormality in hemoglobin may involve all of the following except:

Depletion of one or more amino acids in a globin chain

The pathophysiologic mechanism in acquired idiosyncratic aplastic anemia is:

Destruction of stem cells by autoimmune T cells

The test that is most useful in differentiating FA from other causes of cytopenia is:

Diepoxybutane-induced chromosome breakage

What is a major indication of MDS in the peripheral blood and bone marrow?

Dyspoiesis

What growth factor is produced in the kidneys and is used to treat anemia with kidney disease

EPO

A patient has a platelet count of 700x10^9/L with abnormalities in the size, shape, and granularity of platelets; a WBC count of 12x10^9/L; and hemoglobin of 11 g/dL. The Philadelphia chromosome is not present. The most likely diagnosis is:

ET

A sign of hemolysis that is typically associated with both fragmentation and macrophage-mediated hemolysis is:

Elevated urinary urobilinogen level

The peripheral blood film for patient's with mild HE is characterized by:

Elliptical RBCs

What pathway anaerobically generates energy in the form of ATP?

Embden meyerhof pathway

The myelofibrosis associated with PMF is a result of:

Enhanced activity of fibroblasts owing to increase stimulatory cytokines

Which is true concerning 2,3-BPG?

Enhances O2 release from hemoglobin

The peripheral blood in PV typically manifests:

Erythrocytosis, thrombocytosis, and granulocytosis

A patient has anemia that has been worsening over the last several months. The hemoglobin level has been declining slowly, with a drop of 1.5 g/dl of hemoglobin over about 6 weeks. Polychromasia and anisocytosis are seen on the blood film, consistent with the elevated reticulocyte count and RBC distribution width (RDW). Serum levels of total bilirubin and indirect fractions are normal. Urinary urobilinogen level also is normal. When these findings are evaluated, the conclusion is drawn that the anemia does not have a hemolytic component. Based on the data given here, why was hemolysis ruled out as a cause of the anemia?

Evidence of increased protoporphyrin catabolism is lacking

When a patient has severe anemia and the bone marrow is unable to effectively produce RBCs to meet the increased demand, one of the body's responses is:

Extramedullary hematopoiesis in the liver and spleen

Which of the following describes a penicillin-induced AIHA?

Extravascular hemolysis, positive DAT with IgG, gradual anemia

A patient has a WBC count of 30x10^9/L and the following WBC differential: Segemented neutrophil (38%), bands(17%), metamyelos (7%), myelos (20%), promyelos (10%), eosinophils (3%), basophils(5%): which of the following test results would be helpful in determining whether the patient has CML?

FISH positive for BCR/ABL! fusion

Which one of the following cytokines is required very early in the differentiation of a hematopoietic stem cell

FLT3 ligand

A pronormoblast in its usual location belongs to the RBC mass of the body; but not to the erythron.

False

A patient experiences an episode of acute intravascular hemolysis after taking primaquine for the first time. The physician suspects that the patient may have G6PD deficiency and orders an RBC G6PD assay 2 days after the hemolytic episode begins. How will this affect the test results?

False increase due to reticulocytosis

The clinical consequences of pancytopenia include:

Fatigue, infection and bleeding

Which of the following laboratory tests would be best to confirm PNH?

Flow cytometry for detection of CD55, CD59, and FLAER binding on neutrophils and monocytes

Megaloblastic episodes in SCD can be prevented by prophylactic administration of:

Folic acid

The pathophysiology of idiopathic TTP involves:

Formation of platelt- VWF thrombi due to inhibition of ADAMTS-13

Which of the following is an example of a tranmembrane or intergral membrane protein?

Glycophorin A

Hematopietic stem cells produce all lineages of blood cellsinsufficient quantities over the lifetime of an individual because they:

Have ability of self-renewal by asymmetric division

A 1 year old indian patient presents with anemia, and both parents claim to have an "inherited anemia" but cant remember the type. The peripheral blood shows target cells, and the hemoglobin solubility is negative. Alkaline hemoglobin electrophoresis shows a single band at the "Hb C" position and a small band at the "Hb F" position. Acid hemoglobin electrophoresis shows 2 bands. The most likely diagnosis is:

Hb CO

Which hemoglobinopathy is more common is southeast Asian patients?

Hb E

Which of the following Hb S compound heterozygote exhibits the mildest symptoms?

Hb SG

The process of formation and development of blood cells is termed:

Hematopoiesis

Which of the following is the most definitive test for Hb S?

Hemoglobin electrophoresis at acidic pH

Patients with SCD usually do not exhibit symptoms until 6 months of age because:

Higher levels of Hb F are present

DNA analysis documents a patient has inherited the sickle mutation in both beta-globin genes. The two terms that best describe this genotype are:

Homozygous/disease

In autoimmune hemolytic anemia, a positive DAT is evidence that an:

IgG antibody is senitizing the patient's RBCs

Which of the following is NOT a mechanism of drug-induced hemolytic anemia?

IgM autoantibody sensitization of RBCs after exposure to the cold

The best source of active bone marrow from a 20 year old would be:

Illiac crest

RBC membrane phospholipids are arranged:

In two layers whose composition is asymmetric

The primary pathophysiologic mechanism of anemia associated with chronic kidney disease is:

Inadequate production of erythropeitin

In HS a characteristic abnormality in the CBC results is:

Increased MCHC

Laboratory results for patients with HPP include all the following except:

Increased MCV and normal RDW

The term hemolytic disorder in general refers to a disorder in which there is:

Increased destruction of RBCs after they enter the blood stream

Which of the following results are consistent with HS?

Increased osmotic fragility; negative DAT result

Complications of ET include all of the following EXCEPT:

Infections

Which of the following is a feature found in all macroangiopathic hemolytic anemias?

Intravascular RBC fragmentation

Which one of the following statements is true about DHTR:

It is due to an anamnestic response after repeat exposure to a blood group antigen

The most common mutation found in patients with primary PV is

JAK2 V617F

During the second trimester of fetal development, the primary site of blood cell production is the:

Liver

What single feature of normal RBCs is most responsible for limiting their lifespan

Loss of the nucleus

Chronic myelomonocytic leukemia is classified in the WHO system as:

MDS/MPN

The most consistent peripheral blood findings in severe aplastic anemia are:

Macrocytosis, Thrombocytopenia, and neutropenia

RBC destruction that occurs when macrophages ingest and destroy RBCs is termed:

Macrophage- mediated

Select the statement that is true about bilirubin metabolism

Macrophages of the spleen liberate bilirubin during hemoglobin catabolism

Which of the following determines the timing of egress of RBCs form the bone marrow?

Maturing normoblasts slowly lose receptors for adhesive molecules that bind them to stromal cells

The hemoglobin iron ion may become oxidized to the 3+ valence state by sevral pathological mechanisms. What portion of the embden Meyerhof pathway reduces iron to the physiologic 2+ valence state

Methemoglobin reductase pathway

To survive, the RBC must detoxify peroxides. What hexose monophosphate shunt product(s) accomplishes detoxification?

NADPH and reduced glutathione

Which of the following is least likely to contribute to the death of patients with MDS?

Neuropathy

MDS are most common in which age group?

Older than 50 years

For an erythroid precursor to be considered a ring sideroblast, the iron-laden mitochondria must encircle how much of the nucleus?

One third

Diamond-blackfan anemia differs from inherited aplastic anemia in that in the former:

Only erythropoiesis is affected

Unstable hemoglobins show all of the following findings EXCEPT:

Only homozygotes are symptomatic

Which of the following is the most mature normoblast?

Orthochromic normoblast

Which RBC process does NOT require energy?

Oxygen transport

Which plasmodium species is widespread in Malaysia, has RBCs with multiple ring forms, has band-shaped early trophozoites, shows a 24 hour erythrocytic cycle, and can cause severe disease and high parasitemia

P. knowlesi

Which of the following species of plasmodium produce hypnozoites that can remain dormant in the liver and cause a relapse months or years later?

P. vivax

A patient has a personal and family history of a mild hemolytic anemia. The patient has consistently elevated levels of total and indirect serum bilirubin and urinary urobilinogen. The serum haptoglobin level is consistently decreased, whereas the reticulocyte count is elevated. The latter can be seen as polychromasia on the patient's blood film, along with spherocytes. Which of the findings reported for this patient is inconsistent with a classical diagnosis of fragmentation hemolysis?

Pherocytes on the peripheral film

RBC membrane cholesterol is replenished from the:

Plasma

What erythroid precursor can be describes as follows: the cell is of medium size compared with other normoblasts, with a N:C ratio of nearly 1:1. The nuclear chromatin is condensed and chunky throughout the nucleus. No nucleoli are seen.t he cytoplasm is a muddy, blue-pink color

Polychromatic normoblast

The substitution of valine for glutamic acid at position 6 of the beta chain of hemoglobin results in hemoglobin that

Polymerizes to form tactoid crystals

Mutations in genes that code for the telomerase complex may induce bone marrow failure by causing which one of the following?

Premature death of hematopietic stem cells

Which of the following tests yields results that are abnormal in DIC but are usually within reference interval or just slightly abnormal in TTO and HUS

Prothrombin time and partial thromboplastin time

The most common defect or efficiency in the anaerobic glycolytic pathway that causes chronic HNSHA is:

Pyruvate kinase deficiency

A 63 year old man is being evaluated because of a decrease in hemoglobin of 5 gm/dL after a second cycle of fludarabine for treatment of chronic lymphocytic leukemia. The patient's DAT result is strongly positive for IgGonly, and antibody testing on his serum and an eluate of his RBCs yield positive results with all panel cells and the patient's own cells. This suggests which mechanism of immune hemolysis for this patient?

RBC autoantibody induction

Which of the following tests provide a good indication of accelerated erythropoiesis

Reticulocyte count

What RBC morphology is characteristically found within the first 24 hours following extensive burn injury?

Schistocytes and microspherocytes

Typical laboratory findings in TTP and HUS include:

Schistocytes and thrombocytopenia

RBC membrane block passage of most large molecules such as proetins, but allow passage of small molecules such as the cations, NA+, K+, and Ca2+. What is the term for this membrane property?

Semipermeable

Which of the following sets of test results is typically expected with chronic fragmentation hemolysis?

Serum haptoglobin=increased urine hemoglobin= positive urine sediment prussian blue stain= positive

Abnormalities in the horizontal and vertical linkages of the transmembrane proteins may be seen as:

Shape changes

In hemolysis mediated by IgG antibodies, which abnormal RBC morphology is typically observed on the peripheral blood film?

Spherocytes

Which organ is the site of sequestration of platelets ?

Spleen

Extravascular hemolysis occurs when:

Splenic macrophages ingest senescent cells

The treatment that has shown the best success rate in young patients with severe aplastic anemia is:

Stem cell transplant with an HLA- identical sibling

Hypoxia stimulates RBC production by:

Stimulating EPO production by the kidney

In which of the following geographic areas is Hb S most prevalent?

Sub-Sarahan Africa

A patient presents with mild normochromic, normocytic anemia. On the peripheral blood film, there are a few target cells, rare nRBCs, and hexagonal crystals within and lying outside of the RBCs. Which abnormality in the hemoglobin molecule is most likely?

Substitution of lysine for glutamic acid at position 6 of the beta chain

A 60 year old woman comes to the physician with fatigue and malaise. Her hemoglobin is 8 g/dL, HCT I 25%, RBC count is 2.00x10^12/L, platelet count is 550x10^9/L, and WBC count is 3.8x10^9/L. her WBC differential is unremarkable. Bone marrow shows erythroid hypoplasia and hypolobulated megakaryocytes; granulopoiesis appears normal. Ring sideroblasts are rare. Chromosome analysis reveals the deletion of 5q only. Based on the classification of this disorder, what therpapy would be most appropriate?

Supportive therapy; lenalidomide if the disease progresses

In the bone marrow, RBC precursors are located:

Surrounding macrophages in erythroid islands

Which of the following cells is a product of the CLP?

T lymphocyte

Which of the following chromosome abnormalities is associated with CML?

T(9;22)

A 36 year old woman was brought to the emergency department by her husband because she had experienced a seizure. He reported that she had been well until that morning, when she complained of a sudden headache and malaise. She was not taking any medication and had no history of previous surgery or pregnancy. Laboratory studies showed WBC count 15x10^9/L, hemoglobin level of 7.8g/dL, a platelet count of 18x10^9/L, and schistocytes and helmet cells on the peripheral blood film. Chemistry test results included markedly elevated serum lactate dehydrogenase activity and a slight increase in the level of total and indirect serum bilirubin. The urinalysis results were positive for protein and blood, but there were no RBCs in the urine sediment. Prothrombin time and partial thromboplastin time were within the reference interval. When the entire clinical and laboratory picture is considered, which of the following is the most likely diagnosis?

TTP

An alert hematologist should recognize all of the following peripheral blood abnormalities as diagnostic clues in MDS EXCEPT:

Target cells

Which of the following patterns is characteristic of the peripheral blood in patients with PMF?

Teardrop-shaped erythrocytes, nucleated RBCs, immature granulocytes

A cell has a N:C ratio of 4:1. Which of the following statements would describe it

The bulk of the cell is composed of nucleus

Which of the following is NOT related to the effects of erythropoietin?

The formation of pores in sinusoidal endothelial cells for marrow egress

A group A Rh- negative mother gave birth to a group O Rh- positive baby. The baby is at risk for HDFN if:

The mother was previously immunized to the D antigen

A well mixed specimen obtained for a CBC has a brown color. The patient is being treated with sulfonamide for a bladder infection. Which of the following could explain the brown color?

The patient has Hb M

Which of the following organs is responsible for the maturation of T lymphocytes and regulation of their expression of CD4 and CD8?

Thymus

A patient in whom CML has previously been diagnosed has circulating blasts and promyelocytes the total 30% of leukocytes. The disease is considered to be in what phase>

Transformation to acute leukemia

Intravascular or fragmentation hemolysis os the result of trauma to RBCs while in the circulation

True

Idiopathic acquired aplastic anemia is due to a(n):

Unknown cause

Painful crises in patients with SCD occur as a result of:

Vasooclussion

Based on the criteria in Table 22-1, what is the aplastic anemia classification of a 15 year old female with a bone marrow cellularity of 10%, hemoglobin of 7 g/dL, ANC of 0.1x10^9/L and platelet count of 10x10^9/L

Very severe

A patient has anemia, oval macrocytes, and hypersegmented neutrophils. Which of the following tests would be most efficient in differential diagnosis of this disorder?

Vitamin B12 and folate levels

An elderly white woman is evaluated for worsening anemia, with a decrease of approximately 0.5 mg/dL of hemoglobin each week. The patient is pale, and her skin and eyes are slightly yellow. She complains of extreme fatigue and is unable to complete the tasks of daily living without napping in midmorning and midafternoon. She also tires with exertion, finding it difficult to climb even five stairs. Which of these features of this description points to a hemolytic cause for her anemia?

Yellow skin and eyes