PATHO 370 WEEK 2 (CHAPTER 11) REVIEW QUESTIONS WITH
COMPLETE DETAILED ACCURATE ANSWERS
pe on
1. Classification of Hematologic Neoplasms: Categories based of thethe cell
ty rather than its location in the body. neoplasm
2. B-cell and T-cell combined disorders: -Severe combined immunodeficiency
disorders(SCID)
-Wiskott-Aldrich syndrome
3. Severe combined immunodeficiency disorders (SCID): -Inherited disorde
-Impairment in T and B cells
-Characterized by severe immune system dysfunction and a variety of clinical
features. Most severe form: reticular dysgenesis
4. Wiskott-Aldrich syndrome (WAS): X-linked immunodeficiency disorder that attects
both T cells and B cells
5. T-cell disorders /DiGeorge syndrome: -Is typically due to the deletion of 30 to 40
genes in the middle of chromosome 22 at a location known as 22q11.2.
-About 90% of cases occur due to a new mutation during early development, while 10% are inherited
from a person's parents (autosomal dominant)
-Associated with total or partial loss of thymus gland function
6. DiGeorge syndrome is more often associated with:: -Cardiac
abnormality (commonly interrupted aortic arch, truncus arteriosus and tetralogy of Fallot)
-Abnormal facies
-Thymic aplasia
-Cleft palate
-Hypocalcemia/hypoparathyroidism
7. B-Cell Disorders: -Bruton X-linked agammaglobulinemia.
-IgA deficiency
8. IgA deficiency: -Most common B-cell primary immunodeficiency disorder.
-IgA-bearing lymphocytes fail to become plasma cells, resulting in lack of serum and secretory IgA.
9. Bruton X-linked agammaglobulinemia (XLA): -Congenital
hypogammaglobulinemia
-Lack of normal B-cell development in bone marrow.
-Immunodeficiency disease, failure of B cell maturation to plasma cells; so no plasma cells, antibodies o
germinal centers in lymphoid tissue; recurrent bacterial infections; x-linked inheritance (only males attected);
lyonization protects females who are carriers
1/
8
, PATHO 370 WEEK 2 (CHAPTER 11) REVIEW QUESTIONS WITH
COMPLETE DETAILED ACCURATE ANSWERS
10. Hematologic Neoplasms: Myeloid lineage: red blood cells, platelets,
monocytes, and granu- locytes
11. Hematologic Neoplasms: Lymphoid lineage: B cells, T cells, and natural kill
(NK) cells
12. Malignant Disorders of White Blood Cells: -Leukemia, lymphoma, and
plasma cell myeloma (multiple myeloma)—common neoplastic disorders of the bone marrow and
lymphoid tissues
13. Principles of treatment (Malignand disorders of WBC): -combination
of chemotherapy to remove malignant cells (primary).
-stem cell transplant to rescue and restore bone marrow function (primary).
-radiation and tissue specific drug therapy may be indicated in some cases.
14. Chronic Myeloid Leukemia (CML): -Average age of onset: 40-50 years/Adult
-Characterized by malignant granulocytes that carry the Philadelphia chromosome (Ph+).
15. Philadelphia chromosome (Ph+): Translocation of chromosomes 9 and 22 to
creates a new fusion gene: bcr/abl (increases cell proliferation and reduces apoptotic cell death)
16. CML clinical presentation: -high granulocyte count on the CBC
-splenomegaly
17. Does CML respond well to chemotherapy?: No, poor overall survival time,
untreated patients have median survival 2 years.
18. Acute Myeloid Leukemia (AML): -the most common form of leukemia in adults;
develops when the bone marrow produces too many myeloblasts
19. AML Pathogenesis and Clinical manifestation: -Abrupt onset of
symptoms
-80% of cases are adults; median age 64 years.
-Bone marrow aspirate must have >20% blasts.
-Prognosis worse for AML than ALL: <50% children and 30% adults survive long term.
20. Chronic Lymphoid Leukemia (CLL): Abnormal numbers of relatively mature
lymphocytes predom- inate in the marrow, lymph nodes, and spleen
21. CLL Pathogenesis: -CLL accounts for 30% of all cases of leukemia in the United State
—Most common
-95% are malignant B-cell precursors
-Follows an indolent course; asymptomatic
2/
8
COMPLETE DETAILED ACCURATE ANSWERS
pe on
1. Classification of Hematologic Neoplasms: Categories based of thethe cell
ty rather than its location in the body. neoplasm
2. B-cell and T-cell combined disorders: -Severe combined immunodeficiency
disorders(SCID)
-Wiskott-Aldrich syndrome
3. Severe combined immunodeficiency disorders (SCID): -Inherited disorde
-Impairment in T and B cells
-Characterized by severe immune system dysfunction and a variety of clinical
features. Most severe form: reticular dysgenesis
4. Wiskott-Aldrich syndrome (WAS): X-linked immunodeficiency disorder that attects
both T cells and B cells
5. T-cell disorders /DiGeorge syndrome: -Is typically due to the deletion of 30 to 40
genes in the middle of chromosome 22 at a location known as 22q11.2.
-About 90% of cases occur due to a new mutation during early development, while 10% are inherited
from a person's parents (autosomal dominant)
-Associated with total or partial loss of thymus gland function
6. DiGeorge syndrome is more often associated with:: -Cardiac
abnormality (commonly interrupted aortic arch, truncus arteriosus and tetralogy of Fallot)
-Abnormal facies
-Thymic aplasia
-Cleft palate
-Hypocalcemia/hypoparathyroidism
7. B-Cell Disorders: -Bruton X-linked agammaglobulinemia.
-IgA deficiency
8. IgA deficiency: -Most common B-cell primary immunodeficiency disorder.
-IgA-bearing lymphocytes fail to become plasma cells, resulting in lack of serum and secretory IgA.
9. Bruton X-linked agammaglobulinemia (XLA): -Congenital
hypogammaglobulinemia
-Lack of normal B-cell development in bone marrow.
-Immunodeficiency disease, failure of B cell maturation to plasma cells; so no plasma cells, antibodies o
germinal centers in lymphoid tissue; recurrent bacterial infections; x-linked inheritance (only males attected);
lyonization protects females who are carriers
1/
8
, PATHO 370 WEEK 2 (CHAPTER 11) REVIEW QUESTIONS WITH
COMPLETE DETAILED ACCURATE ANSWERS
10. Hematologic Neoplasms: Myeloid lineage: red blood cells, platelets,
monocytes, and granu- locytes
11. Hematologic Neoplasms: Lymphoid lineage: B cells, T cells, and natural kill
(NK) cells
12. Malignant Disorders of White Blood Cells: -Leukemia, lymphoma, and
plasma cell myeloma (multiple myeloma)—common neoplastic disorders of the bone marrow and
lymphoid tissues
13. Principles of treatment (Malignand disorders of WBC): -combination
of chemotherapy to remove malignant cells (primary).
-stem cell transplant to rescue and restore bone marrow function (primary).
-radiation and tissue specific drug therapy may be indicated in some cases.
14. Chronic Myeloid Leukemia (CML): -Average age of onset: 40-50 years/Adult
-Characterized by malignant granulocytes that carry the Philadelphia chromosome (Ph+).
15. Philadelphia chromosome (Ph+): Translocation of chromosomes 9 and 22 to
creates a new fusion gene: bcr/abl (increases cell proliferation and reduces apoptotic cell death)
16. CML clinical presentation: -high granulocyte count on the CBC
-splenomegaly
17. Does CML respond well to chemotherapy?: No, poor overall survival time,
untreated patients have median survival 2 years.
18. Acute Myeloid Leukemia (AML): -the most common form of leukemia in adults;
develops when the bone marrow produces too many myeloblasts
19. AML Pathogenesis and Clinical manifestation: -Abrupt onset of
symptoms
-80% of cases are adults; median age 64 years.
-Bone marrow aspirate must have >20% blasts.
-Prognosis worse for AML than ALL: <50% children and 30% adults survive long term.
20. Chronic Lymphoid Leukemia (CLL): Abnormal numbers of relatively mature
lymphocytes predom- inate in the marrow, lymph nodes, and spleen
21. CLL Pathogenesis: -CLL accounts for 30% of all cases of leukemia in the United State
—Most common
-95% are malignant B-cell precursors
-Follows an indolent course; asymptomatic
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8
