Systems Pathology II Final Exam- Blood disorders section

Extramedullary hematopoiesis

What is the formation of blood cells outside of the typical medullar bone marrow locations?

Hypovolemic shock

A sudden loss of 20% or more of the total blood volume, which may be due to anemia, can cause what?

Normocytic and normochromic

The red blood cells involved in anemia of blood loss will have what size and color?

Hemolytic

All forms of which type of anemia will cause renal hypoxia, which increases the secretion of EPO, and stimulates erythroid precursors to increase RBC production?

Intracorpuscular

What type of red blood cell defects are most commonly hereditary in nature, and result in weak outer membranes or abnormal hemoglobin formation?

Extracorpuscular

What type of RBC defects are acquired from traumas that injure the outer membrane and include intravascular trauma?

Extravascular

What type of hemolysis occurs within an organ, such as the liver or spleen, due to a defect in the structure of the red blood cells or the binding with an antibody?

True

T/F- Most cases of cholelithiasis are asymptomatic.

Cholesterol

The majority of gallstones are composed of crystalline structures of what molecule?

Pigmented stones

What type of gallstones are composed of unconjugated bilirubin, and develop in people with chronic extravascular hemolysis?

Cholecystitis

What condition will often have a pain referral to the right shoulder?

Biliary colic

What is the term given to pain referred from cholecystitis to the right shoulder?

Fatty foods

What type of foods exacerbate the pain associated with cholecystitis?

Spherocytosis

What condition results from an autosomal dominant defect in the proteins that stabilize the red blood cell's outer membrane, and causes the RBC to take on a spherical shape?

Aplastic crisis (RBC cessation)

What may occur if an individual with spherocytosis acquires parvovirus B19 (fifth disease)?

Sickle cell

What condition develops from an autosomal recessive mutation in the B-globin gene, which results in a sickle-shaped RBC?

Sickle cell

What is the most common form of familial hemolytic anemia, and is described as "unremitting"?

Malaria

Sickle cell disease is most commonly associated with individuals who have been exposed to what mosquito-sourced disease?

Acute chest syndrome and stroke

What are the most common causes of death in an individual with sickle cell anemia?

B thalassemia minor

Which type of thalassemia will result from inheritance of a single B-globin gene mutation, and is often asymptomatic?

B thalassemia major

Which type of B thalassemia results from inheritance of two altered B-globin gene mutations, and follows the absence of B-globin chains within the RBC's hemoglobin?

B thalassemia

Which condition results from mutations to the B-globin gene on chromosome 11 altering the formation of B-globin chains on hemoglobin?

B thalassemia major

Which type of B thalassemia will result in a "hair on end" appearance of the bone structure in the skull, as well as a chipmunk facies?

B thalassemia major

Which B thalassemia condition is treated by repetitive blood transfusions, which can lead to hemochromatosis (iron overloading)?

G6PD

What condition develops following an x-linked recessive disorder that alters the glucose 6 phosphatase enzyme, and leads to a lack of glutathione?

G6PD

Which condition presents in an episodic manner, and may be triggered by consuming fava beans or being around mothballs?

Heinz bodies

What are the clumps of oxidized hemoglobin within the red blood cells of individuals with glucose 6 phosphatase deficiency?

Bite cells

What is the name given to the red blood cells of individuals with G6PD, after splenic macrophages "bite" out the heinz bodies?

PNH (paroxysmal nocturnal hemoglobinuria)

What condition results from an acquired mutation in the x-linked PIGA gene, which suppresses the compliment system?

PNH (paroxysmal nocturnal hemoglobinuria)

Which condition can cause change intravascular hemolysis due to oversensitive complement proteins after subtle changes in blood pH?

PNH (paroxysmal nocturnal hemoglobinuria)

Which condition may cause hemoglobinuria (dark urine) in the morning, which becomes less noticeable as the day progresses?

Traumatic hemolysis

Which blood condition develops from repetitive physical activities, such as a marathon?

Traumatic hemolysis

Which blood condition is most commonly asymptomatic, and produces fragmented red blood cells known as "schistocytes"?

Microangiopathic Hemolytic Anemia

Which condition develops following the narrowing of blood vessels, and causes traumatic hemolysis?

Sporozoites

What form of plasmodium (malaria) effects the hepatocytes of the infected individual?

Merozoites

Which form of plasmodium (malaria) effects the red blood cells of the infected individual?

Gametocytes

Which form of plasmodium is found within the red blood cells of the infected individual, and can actually infect other mosquitos which draw the individual's blood?

Blackwater fever

What is the distinct episodic fever, jaundice, and vomiting caused by the merozoite showers released from lysed blood cells in individuals infected with malaria?

Renal failure

What is the major cause of death in individuals with chronic malaria?

Cerebral malaria

Which form of malaria will involve the CNS, and cause "surface knobs" on the red blood cells to bind vascular endothelium?

Histiolytic neoplasms

What type of neoplasms will involve cancerous cells of the mononuclear phagocyte system, such as macrophages or dendritic cells?

Lymphoid neoplasms

Which type of cellular neoplasms make up the largest group of neoplastic white blood cell disorders, and include all cases of hodgkins/non-hodgkins lymphomas?

Acute lukemias

What cancers are described as having an onset that is "stormy" or aggressive, with anemia being the most common initial feature?

ALL (acute lymphoblastic leukemia)

What is the most common childhood cancer, and the most common childhood leukemia?

ALL (acute lymphoblastic leukemia)

Which cancer will be composed of immature B cells or immature T cells, and may produce superior vena cava syndrome?

ALL (acute lymphoblastic leukemia)

Which cancer involves fewer than 10 mutations, and promotes maturation arrest of B cells or T cells?

T cells

Immature cells of which type are involved in acute lymphoblastic LYMPHOMA, and are found within the thymus?

B cells

Immature cells of which type are involved in acute lymphoblastic LEUKEMIA, and are found within the bone marrow or peripheral blood?

Lymphoma

Which type of ALL will develop later during the pediatric timeframe, around age 15?

CLL (chronic lymphocytic leukemia)

Which cancer is the most common leukemia of adulthood, and affects the mature B cells, altering the regulation of the immune system, resulting in hypogammaglobulinemia?

SLL (small lymphocytic lymphoma)

What cancer is identical to CLL, but is occurs when cancerous B cells are in the lymph structures, rather than the peripheral blood?

Follicular lymphoma

What is a cancer of B cells that are located within the follicular center of an affected lymph node?

follicular lymphoma

What cancer represents 25% of all non-hodgkins lymphoma cases, and contains "centrocytes", which are B cells with "cleaved nuclei"

Follicular lymphoma

Which cancer results from a single mutation translocation between chromosome 14 and 18, which inhibits B cell apoptosis?

Mantle cell lymphoma and follicular lymphoma

Which two cancers uniquely involve lymphadenopathy that is painless?

Mantle cell lymphoma

What is a cancer of the B cells that are located within the mantle zone of affected lymph nodes, and is four times more likely to develop in males?

Mantle cell lymphoma

Which cancer occurs after a mutation to the cyclin D1 gene, which normally regulates the G1 to S phase of the cell cycle?

Mantle cell lymphoma

Which cancer will resemble a colyn polyp mass upon colonoscopy?

Diffuse Large B Cell Lymphoma (DLBCL)

Which cancer involves B cells that are 3-4 times larger than normal, and is the most common lymphoma of adulthood?

Gastrointestinal tract

What the most common extra nodal site for diffuse large B cell lymphoma?

Primary central nervous system lymphoma

Which cancer is an AIDS related diffuse large B cell lymphoma?

Burkitt lymphoma

What condition is common to children living in Africa, and involves the "fastest growing human tumor" on the mandible or maxillae?

Burkitt lymphoma

Which cancer involves an overexpression of the MYC gene, which stimulates the Warburg effect (aerobic glycolysis)?

Burkitt lymphoma

Which cancer may develop from an epstein-barr viral infection, and represents as a "starry-sky" on biopsy?

Multiple Myeloma

What condition consists of cancerous plasma cells with multiple areas of osteolytic destruction throughout the skeleton?

Multiple Myeloma

Which cancer involves immunoglobulins within the blood plasma known as M proteins?

IgG

Most of the immunoglobulins in blood plasma (M proteins) in patients with multiple myeloma are which antibody?

Bence Jones proteins

What are the free light chains in the urine in patients with multiple myeloma?

Spine

What is the most common location for the punched out osteolytic lesions associated with multiple myeloma?

Myeloma nephrosis

What is the renal dysfunction associated with advanced cases of multiple myeloma?

Severe recurrent bacterial infections or kidney failure

What is the most common cause of death in individuals with multiple myeloma?

Hodgkin lymphoma

What lymphoma involves the presence of large Reed-sternberg B cells that have a mirror image "owl eye" appearance?

Hodgkin lymphoma

What type of lymphoma is likely to arise from a single lymph node and spread in a "stepwise" fashion to contiguous nodes?

Hodgkin lymphoma

What cancer is associated with a painless lymphadenopathy of one node, night sweats, and anemia?

Stage I

Which stage of hodgkins lymphoma only involves a single node?

Stage II

Which stage of hodgkins lymphoma involves at least two lymph nodes, and remains on one side of the diaphragm?

Stage III

Which stage of hodgkins lymphoma involves multiple nodes, and crosses both sides of the diaphragm, and often involves the spleen?

Stage IV

Which stage of hodgkins lymphoma has spread to at least one extra lymphatic organ?