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Heme/Onc > WBC Disorders > Flashcards

WBC Disorders Flashcards

1
Q

What is a left shift? What are these cells involved characterized by?

A

It occurs when immature neutrophils are released from the bone marrow in order to respond to infection or necrosis. These immature cells have decreased Fc receptors (CD 16).

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2
Q

What is the only bacteria that shows a high lymphocyte count?

A

Bordetella pertussis

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3
Q

What are the three main organs/sites that EBV infects?

A

Oropharynx (pharyngitis), liver (hepatitis), B cells

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4
Q

What does the CD 8 T cell response in infectious mononucleosis lead to?

A

Generalized LAD, splenomegaly, high WBC count with atypical lymphocytes

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5
Q

What test is used for screening of infectious mononucleosis?

A

Monospot test. Give heterophile antibodies which bind to serum IgM antibodies; test turns positive within 1 week after infection

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6
Q

What test is used for diagnosis of infectious mononucleosis?

A

Serologic testing for EBV viral capsid antigen

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7
Q

What are three complications of infectious mononucleosis?

A
  1. Increased risk for splenic rupture - avoid contact sports for 1 year
  2. Rash with penicillin
  3. Dormancy of virus in B cells - increased risk of recurrence and development of B cell lymphoma especially with HIV
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8
Q

Which cells are the most sensitive to radiation in the human body?

A

Lymphocytes! Most common radiation-induced cancer (after skin) leukemia

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9
Q

What are some clinical features of acute leukemias?

A
  1. > 20% blasts in bone marrow
  2. High WBC count because blasts enter blood
  3. Pancytopenia because blasts crowd out bone marrow - losing normal cells
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10
Q

How do blasts appear on blood smear?

A

Large, immature cells with punched out nucleoli

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11
Q

Which leukemias is Down syndrome associated with?

A

Before Age 5: Acute Megakaryoblastic Leukemia

After Age 5: ALL

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12
Q

Which leukemia requires prophylaxis to scrotum and CSF during chemotherapy?

A

B-ALL; chemotherapy unable to cross blood brain or blood testes barriers

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13
Q

What does T-ALL usually present as?

A

Usually presents in teenagers as a mediastinal/thymic mass –> also called acute lymphoblastic lymphoma
*Remember T T T

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14
Q

AML is usually seen in which group of people?

A

Older adults

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15
Q

How is DIC associated with acute promyelocytic leukemia?

A

The abnormal promyeloblasts that accumulate contain numerous granules. When patients are given ATRA, these cells mature and granules are released; this can lead to DIC

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16
Q

What are myelodysplastic syndromes characterized by?

A

Cells are not being formed properly so are not released into blood –> cytopenia, hypercellular bone marrow, abnormal maturation of cells
Increased blasts but < 20% (can progress to acute leukemia if blasts >20%)

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17
Q

What are some causes of myelodysplastic syndromes?

A

Alkylating agents or radiotherapy

Pre-existing dysplasia

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18
Q

What are some clinical features of chronic leukemia?

A
  1. High WBC count
  2. Increased proliferation of mature circulating lymphocytes
  3. Insidious in onset and in older adults
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19
Q

What is the most common leukemia overall?

A

CLL

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20
Q

Which leukemia co-expresses CD5 and CD20?

A

CLL

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21
Q

What is CLL characterized by?

A
  1. Disease of elderly
  2. Co-express CD5 & CD20
  3. Increased lymphocytes and smudge cells
  4. Can involve lymph nodes and lead to generalized lymphadenopathy - small lymphocytic lymphoma
22
Q

What are three complications of CLL?

A
  1. Hypogammaglobulinemia: neoplastic B cells do not produce Ig
  2. Autoimmune hemolytic anemia: even if they make Ig, do a bad job making it
  3. Richter transformation to diffuse large B cell lymphoma (enlarging lymph node or spleen)
23
Q

Why do you see a dry tap on bone marrow aspiration in hairy cell leukemia?

A

Bone marrow fibrosis

24
Q

What are the clinical features of hairy cell leukemia?

A
  1. TRAP positive stain
  2. Splenomegaly (trapped in red pulp)
  3. NO generalized lymphadenopathy
  4. Dry tap on bone marrow aspiration
25
Q

What are the clinical features of Adult T-Cell Leukemia/Lymphoma?

A
  1. Rash/skin infiltration
  2. Generalized lymphadenopathy
  3. Hepatosplenomegaly
  4. Lytic bone lesions with hypercalcemia
26
Q

What is ATLL associated with?

A

HTLV1; seen in Japan & Caribbean

27
Q

What are the clinical features of mycosis fungiodes?

A

Skin infiltrations - skin rash, plaques, nodules

–> If it goes into the blood called Sezary syndrome

28
Q

What are Pautrier microabscesses?

A

Aggregates of neoplastic T cells in epidermis in mycosis fungiodes

29
Q

What are Sezary cells?

A

Lymphocytes with cerebriform nuclei seen on blood smear; indicates that mycosis fungiodes has entered blood stream

30
Q

Is there a cure for mycosis fungiodes?

A

NO! Fatal lymphoma; can come back

31
Q

What are some characteristics of myeloproliferative disorders overall?

A
  1. High WBC count

2. Cells of all myeloid lineages increased but classified based on dominant myeloid cell produced

32
Q

What are two complications of myeloproliferative disorders?

A
  1. Increased risk for hyperuricemia & gout (increased turnover of cells)
  2. Progression to marrow fibrosis or transformation to acute leukemia
33
Q

Which types of cells are increased in CML?

A

Mature myeloid cells; especially granulocytes and basophils

34
Q

What are complications of CML?

A

Transformation to AML (2/3 of cases) or ALL (1/3 of cases(

* Enlarging spleen indicates accelerated phase of disease

35
Q

What are the clinical symptoms of polycthemia vera due to to? Name 4 symptoms.

A

Hyperviscosity of blood!

  1. Blurry vision and headache
  2. Increased risk of venous thrombosis (Hepatic vein, portal vein, sinus)
  3. Plethora - flushed face
  4. Itching especially after bathing (stimulates mast cells to release histamine)
36
Q

How do you treat polycythemia vera?

A

Phlebotomy; hydroxyurea; reduce risk of vascular events - aspirin

37
Q

How serious is polycythemia vera?

A

Death within a year if untreated!

38
Q

Why is there no significant risk of hyperuricemia or gout in patients with essential thrombocytopenia?

A

Platelets have no nucleus - no nuclear material to turn over

39
Q

What are clinical symptoms of patients with essential thrombocytopenia?

A

Increased risk of bleeding and/or thrombosis or can be asymptomatic

40
Q

Which cells are increased in myelofibrosis?

A

Neoplastic proliferation of mature myeloid cells especially megakaryocytes; produce excess PDGF leading to marrow fibrosis

41
Q

What are clinical features of myelofibrosis?

A
  1. Spleno/hepatomegaly due to extramedullary hematopoiesis
  2. Leukoerythroblastic smear: dacrocytes, nucleated RBCs, immature granulocytes –>
  3. Increased risk of infection, thrombosis and bleeding
42
Q

How can staging and leukemic phase be used to differentiate between Hodgkin and Non Hodgkin lymphoma?

A

Hodgkin - staging guides therapy; no leukemic phase

Non-Hodgkin - staging not very important; leukemic phase usually occurs

43
Q

When do non-Hodgkin lymphomas present? What about Hodgkins?

A

Non-Hodgkin - late adulthood

Hodgkin - young adults

44
Q

What is marginal zone lymphoma associated with?

A

Chronic inflammatory states: Hashimoto thyroiditis, Sjogren syndrome, H pylori gastritis

45
Q

What type of cells does the marginal zone contain?

A

Post-germinal center B cells

46
Q

What is Burkitt’s lymphoma associated with?

A

EBV and HIV

47
Q

What does the release of cytokines by Reed-Sternberg cells result in?

A
  1. B Symptoms: fever, chills, sweats
  2. Attracts reactive lymphocytes, plasma cells, macrophages and eosinophils
  3. Fibrosis may occur
48
Q

What is the most common primary malignancy of bone?

A

Multiple myeloma

49
Q

What cytokine is often present in multiple myeloma?

A

IL-6: stimulates plasma cell growth and Ig production

50
Q

What are clinical features of multiple myeloma?

A

C: hyperCalcemia with bone pain because of activation of RANK receptor on osteoclasts leading to lytic bone lesions
R: Renal failure because of free light chains depositing in kidney tubules
A: Anemia because increased serum protein decreases charge between RBCs causing them to stack
B: Bence Jones protein (light chains) in urine

51
Q

What is Waldenstrom Macroglobulinemia characterized by?

A

B-cell lymphoma with monoclonal IgM production

52
Q

What are four clinical features of Waldenstrom macroglobulinemia?

A
  1. Generalized LAD
  2. M spike (IgM)
  3. Serum hyperviscosity –> visual and neurologic deficits
  4. Bleeding (defective platelet aggregation)

Heme/Onc (3 decks)

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