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Flashcards in WBC malignancies Deck (50)
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1

Myeloid malignancies

arise from myeloid cells, the precursors of granulocytes, RBCs, and megakaryocytes

2

Lymphoid malignancies

arise from lymphoid cells: B lymphocytes (including plasma cells), T lymphocytes, or natural killer cells

3

Leukemia

a malignancy of myeloid OR lymphoid cells in which malignant cells are widespread in BLOOD and BONE MARROW, characterized by a) widespread infiltration of the bone marrow by malignant cells, b) malignant cells in blood, c) proliferation of a clone of malignant cells with a characteristic genetic defect for each type of leukemia, d) complications that include ANEMIA, INFECTION, and HEMORRHAGE due to dysfunctional malignant cells replacing normal RBCs, WBCs, and megakaryocytes.

4

Acute leukemia

Abrupt onset, presenting as acute infection or hemorrhage.

5

acute leukemia s/s

anemia, infection, bleeding, bone pain, enlarged lymph nodes, spleen, and liver. HA, vomiting, nerve palsies. Elevated WBC count (sometimes >100,000), but some cases have WBC counts near normal.

6

Acute leukemia dx

microscopic appearance of blood cells and bone marrow cells, both contain immature WBCs (blasts)

7

Chronic leukemia

Better prognosis than acute leukemia. Ultimately the cause of death is the same with the two leukemias: hemorrhage or infection.

8

Chronic leukemia s/s

fatigue, pallor, anemia, night sweats, low-grade fever, secondary infection, enlarged spleen or liver.

9

Lymphoma

malignancy of the lymphoid cells that grows as masses in lymph nodes or lymph organs: (primary)bone marrow, thymus. Secondary organs: lymph nodes, tonsils, spleen, nodes of GI tract. Not acute or chronic.

10

Myeloid malignancies

leukemias and related disorders that arise from myeloid progenitor cells, which in normal hematopoiesis develop into normal bone marrow and blood cells.

11

Acute myeloid leukemia

Characterized by increased number of myeloblasts in bone marrow and blood. Malignant cells crowd out normal bone marrow and granulocytes, red cells, and megakaryocytes fail to develop. May arise de novo or as a result of other myeloproliferative syndromes. 70% of leukemias. Cigarette smoking is risk factor. Onset is typically sudden.

12

AML s/s

anemia, infection, hemorrhage, bone pain, lymphadenopathy, enlarged spleen and liver, neurologic defects.

13

AML lab values

low RBCs, low granulocytes, low plts

14

AML dx

>20% of bone marrow cells must be myeloblasts.

15

AML tx

chemotherapy. Leukemia that arises from myelodysplasia and myeloproliferative syndromes have a poor prognosis.

16

chronic myeloid leukemia

Rare in children, prevalence increases with age. Characteristic of CML is an abnormally small chromosome 22 and large chromosome 9. Philadelphia chromosome is the small 22. One of the genes produces tyrosine kinase which triggers the production of immature granulocytes. Chemotherapy blocks the production of tyrosine kinase. Progression is slow and has 3 phases. 1) indolent period ~3years, discovery is incidental (via anemia, leukocytosis, enlarged spleen) 2) accelerated phase, characterized by worsening anemia and thrombocytopenia and resistant to treatment, 3) evolution into AML or acute variant of lymphoid leukemia leading to infection or hemorrhage.

17

CML labs

neutrophils, some eosinophils and basophils. High WBC, nucleated RBCs, high plts

18

CML tx

imatinib (Gleevec) gives pts >90% five year survival rate

19

Myelodysplasia syndrome*

Group of hematopoietic progenitor cell disorders that feature ineffective myeloid cell maturation, defective hematopoiesis, and increased risk of AML development. Occur spontaneously or 6-8 years post-chemotherapy/radiation. Bone marrow is occupied by dysplastic cells that do not mature into functional cells. Risk of infection, hemorrhage, anemia.

20

Chronic myeloproliferative syndromes*

One condition with various expressions. All arise from myeloid progenitor cells. They share 3 common tendencies: 1) neoplasia of marrow cells 2) extramedullary hematopoiesis (clinical features are deformed, nucleated RBCs and hepatosplenomegaly) 3) progression into a final, fatal phase featuring marrow fibrosis or acute leukemia. Four disorders: chronic myeloid leukemia, polycythemia vera, malignant thrombocytopenia, primary myelofibrosis with extramedullary hematopoiesis.

21

Polycythemia vera*

Most common myeloproliferative disorder. Almost exclusively affects adults. Malignant transformation of red progenitor cells caused by a specific mutation. May evolve into acute leukemia or myelofibrosis.

22

PV s/s

Tendency for DVT, stroke, hepatosplenomegaly, gout, HTN, pruritis, flushed complexion.

23

PV labs

Increase in RBCs, HCT >60%, increased MCV, increased HGB. PLT and WBC often high.

24

PV dx and tx

Dx: mutation sequencing.
Tx: phlebotomy and aspirin. Chemotherapy avoided

25

Malignant thrombocythemia

(essential thrombocythemia)
Malignant progenitor cells develop toward megakaryocytes. High plts. Leads to thrombosis and hemorrhage. Dx is by exclusion from leukemia, polycythemia, and myelofibrosis

26

Primary myelofibrosis

Malignancy of myeloid cells in which there is non-malignant obliteration of normal bone marrow by fibrous tissue. Scar-like proliferation of fibrous tissue replaces normal marrow. Most malignant cells are in liver and spleen, which rekindle their extramedullary hematopoiesis but this time it's "neoplastic extramedullary hematopoiesis".
Anemia, thrombocytopenia, leukopenia.

27

Acute lymphoid leukemia

Malignant proliferation of immature lymphocyte precursors, usually B cells. Most common malignancy of children. Accumulation of immature blasts in bone marrow that crowd out normal hematopoiesis. Abrupt onset. Malignant cell infiltrations of bone, lymph, nodes, liver, spleen, meninges.

28

ALL s/s

bone pain, lymphadenopathy, hepatosplenomegaly, HA, nerve paralysis, pain.

29

ALL labs

Red cell, granulocyte, PLT all decrease, leading to anemia, infection, and hemorrhage.

30

ALL tx

Chemotherapy very effective, however ALL is the leading cause of cancer death in children.

31

Chronic lymphoid leukemia and SLL

Only difference between CLL and SLL is the number of lymphocytes in the blood (few in SLL, many in CLL). CLL is a malignant proliferation of mature B cells. Most common leukemia. Slow onset.

32

CLL s/s

malaise, mild fever, minimal lymphadnopathy, weight loss, anorexia. Prone to infection (due to malignant B cells that are unable to make antibodies). Lymphocytosis, splenomegaly.

32

CLL s/s

malaise, mild fever, minimal lymphadnopathy, weight loss, anorexia. Prone to infection (due to malignant B cells that are unable to make antibodies). Lymphocytosis, splenomegaly.

33

CLL dx

Lymphocyte count must be >4000, but overall WBC count can vary widely. Bone marrow bx and peripheral smear.

34

CLL tx

should be delayed until symptoms appear. Chemotherapy, steroids, immune therapy, radiation.

35

Hodgkin lymphoma

Distinctive microscopic appearance, tends to arise in a single lymph node, spreads slowly to adjacent nodes, and is mostly curable. People age 10-30. Usually confined to lymph nodes but may infect spleen, liver, bone marrow. Associated with defective cell-mediated (t cell) immunity.

36

HL s/s

cervical lymphadenopathy, fever, night sweats, weight loss, pruritis, splenomegaly, hepatomegaly.

37

Reed-sternberg cell

B lymphocyte of HL. Becomes malignant because of Epstein Barr Virus. There are 5 variants, but nodular sclerosing Hodgkin lymphoma is the variant that accounts for about 70% of cases, which disproportionately affect women.

38

HL dx and tx

Dx: lymph node bx. Clinical staging is important.
Tx: chemotherapy w/ or w/o radiation

39

Non-Hodgkin lymphoma

80% are malignant tumors of B lymphocytes. Like HL, both are tumors of lymphocytes, present as painless enlargement of lymph nodes, and associated with immune deficiency and infection. More aggressive. Many arise in organs outside the lymph node, such as brain, bone or bowel. Spreads widely throughout body. Clinical staging not as important as HL.

40

NHL s/s

enlarged, non-tender lymph nodes, fever, weight loss, malaise, sweating, autoimmune pna or immunodeficiency problems that lead to infection.

41

Follicular lymphoma

NHLs with follicular microscopic appearance. Growth pattern is similar to the lymphoid follicles of normal lymph nodes. Less aggressive. Do not respond well to chemotherapy.

42

Diffuse lymphoma

Grow without follicles. Mostly people over 60, but also occur in childhood and those with AIDS. Most grow rapidly and are quickly lethal, but are somewhat responsive to treatment.

43

Multiple myeloma

-Malignant plasma cells
-PUNCHED OUT bone defects
-hypoimmunoglobulinemia
-impaired humoral immunity
Malignant neoplasm of plasma cells that features multiple bone lesions. May be preceded by solitary myeloma. Peak age = 65y. Malignant plasma cells don’t circulate blood; they are masses in the bone marrow. They destroy bone and produce “punched out” bone defects. Antibody production is affected, leading to hypogammaglobulinemia and subsequent susceptibility to infections due to impaired humoral immunity.

44

multiple myeloma s/s

bone pain, hypercalcemia (d/t bone destruction), anemia, recurrent infections, kidney failure due to Bence-Jones proteins.

45

Bence-Jones protein

Free light chain portions of immunoglobulins that have been corrupted. Excreted in urine, but produce a toxic effect on renal tubules and are present in multiple myeloma

46

multiple myeloma dx and tx

Dx: punched out bone on radiograph
Tx: chemotherapy, radiation, survival 3-5y

47

Lymphoplasmacytic lymphoma - Plasma cell overproduction of IgM

B cell neoplasm of older adults, related to CLL/SLL. The malignant cells differentiate into plasma cells that secrete IgM. When present in excess, IgM thickens plasma into a syrupy, viscous fluid, causing sluggish blood flow

48

Waldenstrom macroglobulinemia

Hyper viscosity syndrome. Caused by lymphoplasmacytic lymphoma. Symptoms similar to multiple myeloma, but bone lesions do not occur.
-Impaired blood flow to brain = dizziness, HA, confusion, stroke, visual problems.
-Hemorrhage (bc IgM interferes with clotting factors.

49

nodular sclerosing hodgkin lymphoma

comprises 70% of cases. disproportionately affects women. affects neck and chest lymph nodes