hematology everything

beta, gamma, and epsilon hemoglobin chain loci are found on which chromosome

11

Alpha and zeta hemoglobin loci are found on which chromosome?

16

How does hydroxyurea aid in the treatment of sickle cell disease?

Induces increased production of HbF. Hydroxyurea induces increased production of HbF. Most sickle cell patients who have increased levels of HbF experience milder forms of the disease than do patients with normal or low levels of HbF. Therefore, the focus of molecular treatments for sickle cell disease is to increase fetal hemoglobin (HbF).

Which of the following laboratory test results is not usually increased in alpha thalassemia? Iron binding capacity Percent iron saturation Lactate dehydrogenase Bilirubin

Iron binding capacity

In almost all cases of polycythemia vera, which genetic abnormality is present? hint: mutation

JAK2 mutation

Nitric oxide is associated with the prevention of vaso-occlusion by decreasing cellular adherence to endothelium. Which amino acid is decreased in patients with sickle cell disease and is needed as a substrate to produce nitric oxide?

L-arginine The amino acid L-arginine is a substrate needed to produce nitric oxide. Low arginine bioavailability in sickle cell disease is associated with elevated levels of nitric oxide synthase inhibitor, among other complications.

Which anomaly is associated with Dohle body-like inclusions (large, blue-staining cytoplasmic inclusions), as well as bizarre/giant platelets.

May-Hegglin anomaly

Newborns who will develop Hemoglobin H disease may demonstrate up to 30% of which type of hemoglobin?

Barts

What is the prominent red blood cell (RBC) morphologic feature found in lead poisoning (plumbism)?

Basophilic stippling

Cooley's anemia is another name for which blood disorder?

Beta thalassemia major

In what order do normal and abnormal hemoglobins migrate in alkaline electrophoresis (from cathode to anode)? H C F S A A2

C with A2, S, F, A, H

Cooley's anemia/beta-thalassemia major

Clinical symptoms usually develop during an affected child's first year of life. The child appears to be malnourished and may exhibit abdominal girth expansion. Skeletal deformations are evident, which are a result of increased erythropoiesis. A common finding is facial bone changes. Other clinical signs include frequent infections, hepatomegaly, splenomegaly, cardiomegaly, gall stones, leg ulcers, and poor growth and sexual development. Death usually occurs by the time these patients are in their early twenties unless treated with blood transfusions along with iron-chelating agents

Which of the following constitutes the alpha hemoglobin chain coding? A. Two gene loci; one each on chromosome 11 B. Four gene loci; two each on chromosome 11 C. Two gene loci; one each on chromosome 16 D. Four gene loci; two each on chromosome 16

D. Four gene loci; two each on chromosome 16

Alpha thalassemia is defined as:

Decrease in the rate of production of Alpha chains due to partial or total deletion of the genetic code

Chédiak-Higashi syndrome

Defect: - LYST Mutation - *Lysosomal Trafficking (LYST) Mutation* - Defect in *Phagolysosome* Formation - Smear: *Giant Granules in Neutrophils* (Not Phagocytosed) Symptoms: - *Pyogenic Infections* - *Albinism* - *Neuropathy* represents a qualitative disorder of neutrophils. It is a rare disorder this expressed as an abnormal granulation of neutrophils. Neutrophils with giant granules display impaired chemotaxis and delayed killing of ingested bacteria.

What condition would be suspected in an asymptomatic patient with greater than 20% atypical (reactive) lymphocytes on their peripheral blood smear? Herpes simplex virus infection Epstein-Barr virus infection Bacterial meningitis Acute leukemia

Epstein-Barr virus infection

The autohemolysis test is MOST useful in the diagnosis of which condition?

Hereditary spherocytosis

The bone marrow in alpha thalassemia major usually demonstrates: hyperplasia or hypoplasia

Hypoplasia

Hemoglobin H bodies can directly alter the integrity of which cell membrane

RBC compromised when macrophages remove the precipitated tetramers out of the cell. These cells can appear as if "bites" were taken out of them or as fragmented cells (schistocytes).

Alpha thalassemia minor

Reduced alpha-globin production anemia: mild to absent. RBCs : increased HGB : slight decrease MCV: slight decrease MCHC: slight decrease RDW: slightly increased Mild anemia Microcytic RBCs Target cells on blood smear

Which one of the following is a useful chemical test for the diagnosis of hairy cell leukemia? Peroxidase test Sudan black test Alpha-naphthyl acetate esterase test Tartrate-resistant acid phosphatase test

Tartrate-resistant acid phosphatase test (TRAP)

What are the usual results of the mean corpuscular volume (MCV) and the red cell distribution width (RDW) in alpha thalassemia?

The MCV is decreased but the RDW is usually increased

What is a typical finding for determining the endpoint for the initial or iron-depletion phase of treatment for hereditary hemochromatosis (HH)?

The serum ferritin decreases to between 20 and 50 ng/mL

The gene loci for the alpha globin chains are adjacent to the locus for which other globin chain?

Zeta

Increased __________ is most responsible for increasing the ESR. __________ and other similar proteins decrease the zeta potential or negative charge of red cells, allowing them to come closer together and sediment more rapidly.

fibrinogen, fibrinogen

why does haptoglobin decrease in alpha thallasemia?

haptoglobin functions by binding the alpha chain portion of hemoglobin. With the absence of these chains in alpha thalassemia major and intermedia, haptoglobin cannot bind free hemoglobin. Therefore it is not consumed as it would be in other types of hemolytic anemia

What are the globular inclusions in this cell? hint: Prominent cell is a Mott cell on a peripheral blood smear from a patient with multiple myeloma.

immunoglobulin accumulations (Russell bodies), representing the accumulation of excessive mucopolysaccharides and globulins within the endoplasmic reticulum.

hemoglobin H morphology

inclusions are faint blue and appear to be on the outside of the cell, resembling sugar on a gumdrop; but they are just inside the cell's membrane and push outward (Cell C in image)

glitter cell

neutrophil seen in dilute/hypotonic urine usually with sparkling appearance.

Myeloperoxidase (MPO) AML result

positive

Sudan Black stain AML result

positive

Hb Portland

an embryonic hemoglobin having 2 gamma and 2 zeta chains.

Gaucher's disease

rare genetic defect of macrophages. In this disorder, a deficiency of ß-glucocerebrosidase, the enzyme that normally splits glucose from its parent sphingolipid, glucosylceramide. As a result of the enzyme deficiency, cerebroside accumulates in macrophages. These cells are rarely seen in circulating blood but are found in the bone marrow, spleen, and other organs of the mononuclear phagocyte system.

When would Döhle bodies be present in a blood smear?

sepsis, bacterial infections, and severe burns.

Erythropoietin is mainly produced in:

the kidneys