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Pathology Block 14 > White Blood Cell Disorders > Flashcards

White Blood Cell Disorders Flashcards

1
Q

Most common lymphoid neoplasms?

A

B cell origin

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2
Q

ALL lab findings:
___penia
___
Platelet count?

A

Neutropenia
Anemia
< 100,000

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3
Q

Age ranges:

  1. B-ALL
  2. T-ALL
A
  1. Peak age is 4 (over 5 if patient has down syndrome)

2. 15-20

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4
Q

Location:

  1. B-ALL
  2. T-ALL
A
  1. Bone marrow/peripheral blood

2. Thymus masses

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5
Q

Translocation and feature that predict good outcome of B-ALL

A

T(12;21)

Hyperdiploidy (>50 chromosomes per cell)

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6
Q

Poor prognosis for B-ALL

A

Philadelphia chromosome - t(9;22)

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7
Q
  1. What are Auer rods
  2. What do they look like
  3. What disorder are they seen in
A
  1. Crystal aggregates of MPO (in granulocytes)
  2. Red staining, needle like structures
  3. Acute myeloid leukemia
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8
Q
  1. t(15;17) is translocation for which disorder

2. What does this disorder have a risk for?

A
  1. Acute promyelocytic leukemia

2. DIC because this disorder produces a lot of Auer rods

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9
Q
  1. What disorder lacks MPO but is lysosomal nonspecific esterase positive?
  2. What do the nuclei look like in this disorder?
  3. Classic presentation?
A
  1. Acute monocytic leukemia
  2. Crumpled tissue paper
  3. Gingival leukocytic infiltrates
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10
Q

Acute megakaryoblastic leukemia is associated with ?

A

Down syndrome (BEFORE 5 years old)

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11
Q

What is the most common adult leukemia?

A

Chronic lymphocytic leukemia / small lymphocytic lymphoma

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12
Q

How to determine between CLL and SLL?

A

If lymphocytosis is >4,000 then it is CLL; if not it is SLL

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13
Q

What type of cells will you see on peripheral blood smear of CLL/SLL?

A

Smudge cells (will look like they popped due to weaker cell membranes)

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14
Q
  1. Unique marker of CLL/SLL?

2. Which disorder also has this same marker?

A
  1. CD5

2. Mantle cell lymphoma

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15
Q

What is the most common cause of death in CLL/SLL? Why?

A

Hypogammaglobulinemia because it increases risk of bacterial infection

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16
Q

Chronic mylogenous leukemia is a disorder of what type of cell

A

Pluripotent stem cell

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17
Q
  1. Translocation for CML?

2. Acquired genetic abnormal gene for CML?

A
  1. t(9;22)

2. BCR-ABL fusion gene

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18
Q

Unique morphology?

A

Prominent basophils and eosinophils

Also thrombocytosis since platelets are also from myeloid cell line

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19
Q

Age range for CML

A

25-60 years old

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20
Q

Clinical feature of CML

A

Extreme splenomegaly

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21
Q

How to differentiate between CML and lymphoid reaction

A

Leukocyte alkaline phosphatase (LAP) will be negative in CML

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22
Q

Hairy cell leukemia

  1. Who is most affected?
  2. Mutation in ?
  3. Clinical features
  4. Prognosis?
A
  1. Older men
  2. BRAF gene
  3. Splenomegaly; lymphadenopathy is usually absent
  4. Excellent
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23
Q

Most common type of cutaneous T cell lymphoma?

A

Mycosis fungoides/sezary syndrome

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24
Q

Sezary cells have __ nuclei

A

Cerebriform

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25
Q

How do you differentiate between cutaneous T cell lymphoma and adult T cell leukemia?

A

Adult T cell leukemia expresses CD25

26
Q

What symptom should make you think of leukemia?

A

Painless lymphadenopathy

27
Q

Cells seen in Hodgkin lymphoma

A

Reed-Sternberg cells (owl eye)

28
Q

2 positive CD markers in hodgkin lymphoma

A

CD15 and CD30

29
Q

Hodgkin lymphoma:

  1. Why is it different than other B cell lymphomas
  2. Ages affected
  3. 50% of cases are associated with
  4. Common location of enlarged lymph nodes
A
  1. Spreads to adjacent node groups (no extranodal spread or leukemic state)
  2. Affects the young and the old
  3. EBV
  4. Mediastinum
30
Q
  1. Most common form of Hodgkin lymphoma
  2. Has __ variant of reed-sternberg cells
  3. Commonly affects what age
  4. 3 main lymph node locations
A
  1. Nodular sclerosis (so when in doubt choose this one)
  2. Lacunar
  3. Adolescents and young adults
  4. Mediastinum, lower cervical and supraclavicular
31
Q
  1. Second most common type of hodgkins
  2. Age affected
  3. Lots of what cell type on smear?
A
  1. Mixed cellularity
  2. > 50 (usually males)
  3. Eosinophils
32
Q
  1. Which hodgkins as the best prognosis of all types?
  2. 2 locations of lymph nodes
  3. __ variant of reed-sternberg cells
A
  1. Lymphocyte-predominant
  2. Isolated: either cervical or axillary
  3. Lymphohistiocytic (popcorn)
33
Q

Staging of lymphoma

  1. Stage I
  2. Stage II
  3. Stage III
  4. Stage IV
A
  1. Single lymph node region involved
  2. Involvement of 2 or more lymph nodes on the same side of the diaphragm
  3. Involvement of lymph nodes on BOTH sides of the diaphragm
  4. Multiple/disseminated foci of one or more extralymphatic organs/tissues with or without lymphatic involvement
34
Q
  1. Most common indolent non-hodgkins lymphoma
  2. Translocation
  3. Translocation leads to expression of __
  4. __ is almost always involved at the time of diagnosis
A
  1. Follicular lymphoma
  2. t(14;18)
  3. BCL2 (which prevents apoptosis)
  4. Bone marrow
35
Q

Mantle cell lymphoma:

  1. Usually affects what type of person
  2. Frequent involvement of __
  3. Similarity to CLL/SLL?
  4. Translocation - has what effect?
  5. Prognosis?
  6. __ cells
A
  1. Older males
  2. GI tract
  3. CD5 expression
  4. t(11;14) -> dysregulates cyclin D1 (so increases cell proliferation)
  5. Poor - aggressive and incurable
  6. Buttock cells
36
Q
  1. Most common and aggressive NHL?
  2. Patient presentation
  3. Which 3 organs are usually NOT involved at time of diagnosis
  4. Describe cells seen on smear
A
  1. Diffuse large B cell lymphoma
  2. GI tract and brain issues
  3. Liver, spleen and bone marrow
  4. Large nuclei with open chromatin and prominent nucleoli
37
Q

3 subtypes of diffuse large B cell lymphoma

A
  1. EBV (AIDS/post-transplant)
  2. HHV-8 (Kaposis sarcoma)
  3. Mediastinal large B cell lymphoma
38
Q

Burkitt lymphoma:

  1. Morphology?
  2. Translation (3)
  3. Age affected
  4. Common presentation in US
A
  1. Starry sky (basophilic cytoplasm with lipid vacuoles
  2. t(8;14), t(2;8), t(8;22) - MYC is on chromosome 8
  3. Children and young adults
  4. Abdominal tumors
39
Q

How to differentiate burkitt lymphoma from ALL

A

Burkitt lymphomas are fast growing and there is a mass present

40
Q

Extranodal marginal zone lymphoma arises in what setting

A

Autoimmune disorders or chronic infection

41
Q
  1. Most common malignant plasma cell dyscrasia?
  2. Peak age
  3. Presentation?
A
  1. Multiple myeloma
  2. 70
  3. Multifocal lytic lesions throughout the skeletal system
42
Q

Multiple myeloma:

  1. Associated with what proteins
  2. M component is typically __ or __
  3. What does it look like on smear?
  4. Associated with what electrolyte abnormality?
  5. 2 most common causes of death
A
  1. Bence jones proteins in urine
  2. IgG or IgA
  3. Rouleaux formations (stacked like coins)
  4. Hypercalcemia (from bone breakdown)
  5. Recurrent infections; renal insufficency
43
Q

Lymphoplasmacytic lymphoma:

  1. How is it different from CLL/SLL?
  2. How is it different from multiple myeloma
A
  1. Many of the tumor cells undergo differentiation to plasma cells
  2. No free light chains or lytic bone lesions
44
Q

M component of lymphoplasmacytic lymphoma

A

IgM - so causes hyperviscosity and worse in cold temperatures

45
Q

Waldenstrom macroglobulinemia - 2 main symptoms

A

Visual impairment and neurologic problems

46
Q

Common presentation of primary amyloidosis

A

“Raccoon eyes”, deposits on the tongue, peripheral neuropathy, swollen hands/feet

47
Q

Monoclonal gammopathy of undetermined significance presentation

A

Asymptomatic - usually found accidentally

48
Q

Myelodysplastic syndromes and chronic myeloproliferative disorders can often transform to __

A

AML

49
Q

Myelodysplastic syndromes are characterized by __ defects and have a high risk of transforming to __

A

Maturation; AML

50
Q

2 common abnormalities in myelodysplastic syndromes

A

Megaloblastoid erythroid precursors (like megaloblastic anemias) and erythroid forms with ring sideroblasts

51
Q
  1. Myelodysplastic syndromes have a loss of _ or _
  2. Age range
  3. Worse prognosis if __ deletion
  4. Risk factor
A
  1. 5 or 7
  2. 50-70 years old
  3. P53
  4. Previous chemo
52
Q

What type of mutations are common in chronic myeloproliferative disorders

A

Tyrosine kinases

53
Q

Polycythemia vera has proliferation of which 3 elements

A

Erythroid, granulocytic, and megakaryocytic (all myeloid cell line)

54
Q

Relative polycythemia vs reactive polycythemia

A

Low levels of erythropoietin in polycythemia vera

55
Q

Polycythemia vera:

  1. Patient presentation
  2. What is in peripheral blood
A
  1. Liver and spleen enlargement, red hue to the skin, itching, bleeding (due to extra platelets)
  2. Basophilia
56
Q

Essential thrombocythemia

  1. Proliferation of ?
  2. Mutation
  3. Risk of
A
  1. Platelets (mostly), RBCs and granulocytes
  2. JAK2 kinase
  3. Risk of thrombosis/bleeding
57
Q

Primary myelofibrosis:

  1. What happens early in disease?
  2. Mutation
  3. What do the cells look like
  4. Release of what 2 factors
  5. Symptoms
A
  1. Marrow fibrosis
  2. JAK2
  3. Teardrops
  4. PDGF and TGF beta
  5. Basophilia, hyperuricemia (gout), RBCs with abnormal shapes
58
Q

Langerhans cell histiocytosis

  1. Main markers
  2. __ in the cytoplasm - what do they look like
  3. Mutation
  4. Found mostly in the __
A
  1. HLA-DR, CD1a, S100, langerin
  2. Birbeck granules - tennis racket shaped
  3. BRAF (RAS signaling pathway)
  4. Skin
59
Q

Multisystem langerhans cell histiocytosis (Letterer-Siwe disease)

  1. Age
  2. Symptoms
  3. Prognosis
A
  1. < 2 years old
  2. Recurrent infections (ear and mastoid), destructive bone lesions, cutaneous lesions, pulmonary lesions
  3. Fatal
60
Q

Unisystem langerhans cell histiocytosis (eosinophilic granuloma)

  1. Location of lesions
  2. Histiocytes mixed with what other 4 cell types
A
  1. Medullary cavities of the bone, skin, lungs, stomach

2. Eosinophils, lymphocytes, plasma cells and neutrophils

61
Q

Unisystem langerhans cell histiocytosis (eosinophilic granuloma)

  1. Unifocal lesion presentation
  2. Multifocal lesion presentation
A
  1. Usually skeletal; asymptomatic

2. Children, fever, URIs; hand-schuller-christian disease

62
Q

Hand-schuller-christian disease: 3 symptoms

A
  1. Calvarial bone defects
  2. Diabetes insipidus
  3. Exophthalmos

Pathology Block 14 (1 decks)

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