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Flashcards in Intro to White Blood Cell Disorders Deck (61)
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1

Where are WBCs found?

  • Bone marrow
  • Peripheral blood
  • Lymph nodes, thymus, spleen, tonsils, adenoids, Peyer patches
  • Mucosa-associated lymphoid tissue (MALT)

2

What is cytoses?

What is cytopenia?

Increased leukocytes

Decreased leukocytes

3

What is the normal reference range for WBCs?

4000 - 10,000/uL

4

What is a leukemoid reaction and what cells may be involved?

NOT leukemia

A benign, exaggerated response to infection with an absolute leukocyte count > 50,000

May involve neutrophils, lymphocytes or eosinophils

5

What are potential causes of a leukemoid reaction?

  • Perforating appendicitis - neutrophils
  • Whooping cough (Bordetella pertussis) - lymphocytes
  • Cutaneous larva migrans - Eosinophils

6

What is a leukoerythroblastic reaction? 

What can cause it?

Immature bone marrow cells in the peripheral blood

Can be due to a BM infiltrative disease (fibrosis or breast cancer metastasis) or severe BM stress (sepsis or growth factor)

7

Neutrophilia is defined as an absolute neutrophil greater than ________

7,000/uL

8

What are some causes of neutrophilia?

  • Infection
  • Sterile inflammation with necrosis (acute MI)
  • Drugs (steroids, catecholamines, lithium)
  • Increased production and decreased margination (extravasation)

9

Neutropenia is an absolute neutrophil count less than ______

1500/uL

10

What are some possible causes of neutropenia?

  • Chemotherapy
  • Aplastic anemia
  • Immune destruction
  • Septic shock
  • Decreased production and/or increased destruction or margination

11

Eosinophilia is an absolute eosinophil count greater than _____ and is caused by...(4)

700/uL

  • Type I hypersensitivity (allergies)
  • Invasive helminths (hookworm) 
  • Hypocortisolism (Addison's disease)
  • Neoplasms (Hodgkin lymphoma)

12

Basophilia is a basophil count greater than ____ and can be caused by... (2)

200/uL

  • Chronic myelogenous leukemia (and other chronic myeloproliferative neoplasms)
  • Chronic kidney disease

13

What do leukemia and lymphoma have in common?

What is the difference?

Both are a proliferation of neoplastic cells

Leukemia: Primarily in BM and PB (peripheral blood)

Lymphoma: Primarily in lymph nodes and extramedullary lymphoid tissue

14

What four criteria are used to classify Myeloid neoplasms (neoplastic stem cell disorders)

  • Morphology
  • Immunophenotype
  • Genetic features
  • Clinical features

15

What are the 3 categories of myeloid neoplasms?

  1. Myeloproliferative neoplasms (MPN)
  2. Myelodysplastic syndromes (MDS)
  3. Acute myeloid leukemia (AML)

16

What are four types of myeloproliferative neoplasms?

  1. Chronic myelogenous leukemia, BCR-ABL positive (CML)
  2. Polycythemia vera (PV)
  3. Primary myelofibrosis (PMF)
  4. Essential thrombocythemia (ET)

17

MPN is a proliferation of...

One or more of the myeloid lineages

  • Granulocytic
  • Erythroid
  • Megakaryocytic
  • Mast cells

18

MPN general features

  • Age group:
  • BM appearance:
  • Effect on other organs:
  • Possible complications:

  • Age group: Common in adults (5th - 7th decade)
  • BM appearance: Hypercellular BM with effective hematopoiesis
  • Effect on other organs: Splenomegaly or hepatomegaly
  • Possible complications: Potential for progression - BM fibrosis or acute leukemia

19

What are the differences between myeloproliferative neoplasms (MPN) and myelodysplastic syndromes (MDS)?

  • MPN: Hypercellular BM with effective hematopoiesis (cytoses, increased PB counts, cell proliferation)
  • MDS: Hypercellular BM with ineffective hematopoies-is (cytopenias, decreased PB counts, cell death)

 

20

Chronic myelogenous leukemia (CML)

  • Age group:
  • Cellular mechanism:
  • Genetic defect:
  • Clinical findings:

  • Age group: peak at 40-60 years
  • Cellular mechanism: Neoplastic expansion of pluripotential stem cell
  • Genetic defect: BCR-ABL fusion gene
  • Clinical findings: Hepatosplenomegaly; weight loss, fatigue, weakness, anorexia

21

What are the laboratory findings in CML?

  • Leukocytosis with immature myeloid cells
  • Basophilia
  • Philadelphia chromosome: t(9;22)
  • BCR-ABL fusion gene
  • Few myeloblasts
  • Thrombocytosis (45-50%) or thrombocytopenia
  • Hypercellular BM (granulocytic hyperplasia)

22

What are the 3 stages in the course of CML?

  1. Chronic phase (3 years)
  2. Accelerated phase (1 year)
  3. Blast phase = acute leukemia (myeloid or lymphoblastic)

23

What therapy is done for CML?

  • Allogeneic stem cell transplant
  • BCR-ABL tyrosine inase inhibitors
    • Gleevec
    • Dasatinib
    • Nilotinib

24

Polycythemia vera (PV)

  • Increase in...
  • Genetic defect:

  • Increase in RBCs, granulocytes and platelets
  • Genetic defect: Janus 2 kinase (JAK2) mutation in virtually all cases

25

Clinical findings in PV

 

  • Splenomegaly
  • Thrombotic events (due to hyper viscosity)
  • Gout (increased cell breakdown)
  • Increased histamine (from mast cell sin the skin)
    • Ruddy face, pruritus after bathing, peptic ulcer

26

What are the laboratory findings in PV?

  • Increased RBC mass (increased hemoglobin)
  • Decreased EPO (dysfunctional Hb feedback loop)
  • Leukocytosis
  • Thrombocytosis
  • Normal oxygen saturation
  • Hypercellular BM with fibrosis (in later stages)

27

EPO increases in all polycythemias EXCEPT...

Polycythemia vera

28

With conservative treatment of PV, median survival is greater than ___

Most patients die of _____ or ______

Median survival is greater than 10 years

Most patients die of thrombosis or hemmorhage

29

Primary myelofibrosis (PMF) involves rapid development of __ _______ and  _______ _______ in the spleen, liver and lymph nodes

BM fibrosis; Extramedulary hematopoiesis (EMH)

30

What are the clinical findings in PMF? (2)

  • Splenomegaly (with portal hypertension)
  • Splenic infarcts with left sided pleural effusions