White Blood Cell Disorders Flashcards
1
Q
Most common lymphoid neoplasms?
A
B cell origin
2
Q
ALL lab findings:
___penia
___
Platelet count?
A
Neutropenia
Anemia
< 100,000
3
Q
Age ranges:
- B-ALL
- T-ALL
A
- Peak age is 4 (over 5 if patient has down syndrome)
2. 15-20
4
Q
Location:
- B-ALL
- T-ALL
A
- Bone marrow/peripheral blood
2. Thymus masses
5
Q
Translocation and feature that predict good outcome of B-ALL
A
T(12;21)
Hyperdiploidy (>50 chromosomes per cell)
6
Q
Poor prognosis for B-ALL
A
Philadelphia chromosome - t(9;22)
7
Q
- What are Auer rods
- What do they look like
- What disorder are they seen in
A
- Crystal aggregates of MPO (in granulocytes)
- Red staining, needle like structures
- Acute myeloid leukemia
8
Q
- t(15;17) is translocation for which disorder
2. What does this disorder have a risk for?
A
- Acute promyelocytic leukemia
2. DIC because this disorder produces a lot of Auer rods
9
Q
- What disorder lacks MPO but is lysosomal nonspecific esterase positive?
- What do the nuclei look like in this disorder?
- Classic presentation?
A
- Acute monocytic leukemia
- Crumpled tissue paper
- Gingival leukocytic infiltrates
10
Q
Acute megakaryoblastic leukemia is associated with ?
A
Down syndrome (BEFORE 5 years old)
11
Q
What is the most common adult leukemia?
A
Chronic lymphocytic leukemia / small lymphocytic lymphoma
12
Q
How to determine between CLL and SLL?
A
If lymphocytosis is >4,000 then it is CLL; if not it is SLL
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)
14
Q
- Unique marker of CLL/SLL?
2. Which disorder also has this same marker?
A
- CD5
2. Mantle cell lymphoma
15
Q
What is the most common cause of death in CLL/SLL? Why?
A
Hypogammaglobulinemia because it increases risk of bacterial infection
16
Q
Chronic mylogenous leukemia is a disorder of what type of cell
A
Pluripotent stem cell
17
Q
- Translocation for CML?
2. Acquired genetic abnormal gene for CML?
A
- t(9;22)
2. BCR-ABL fusion gene
18
Q
Unique morphology?
A
Prominent basophils and eosinophils
Also thrombocytosis since platelets are also from myeloid cell line
19
Q
Age range for CML
A
25-60 years old
20
Q
Clinical feature of CML
A
Extreme splenomegaly
21
Q
How to differentiate between CML and lymphoid reaction
A
Leukocyte alkaline phosphatase (LAP) will be negative in CML
22
Q
Hairy cell leukemia
- Who is most affected?
- Mutation in ?
- Clinical features
- Prognosis?
A
- Older men
- BRAF gene
- Splenomegaly; lymphadenopathy is usually absent
- Excellent
23
Q
Most common type of cutaneous T cell lymphoma?
A
Mycosis fungoides/sezary syndrome
24
Q
Sezary cells have __ nuclei
A
Cerebriform
25
How do you differentiate between cutaneous T cell lymphoma and adult T cell leukemia?
Adult T cell leukemia expresses CD25
26
What symptom should make you think of leukemia?
Painless lymphadenopathy
27
Cells seen in Hodgkin lymphoma
Reed-Sternberg cells (owl eye)
28
2 positive CD markers in hodgkin lymphoma
CD15 and CD30
29
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
1. Spreads to adjacent node groups (no extranodal spread or leukemic state)
2. Affects the young and the old
3. EBV
4. Mediastinum
30
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
1. Nodular sclerosis (so when in doubt choose this one)
2. Lacunar
3. Adolescents and young adults
4. Mediastinum, lower cervical and supraclavicular
31
1. Second most common type of hodgkins
2. Age affected
3. Lots of what cell type on smear?
1. Mixed cellularity
2. > 50 (usually males)
3. Eosinophils
32
1. Which hodgkins as the best prognosis of all types?
2. 2 locations of lymph nodes
3. __ variant of reed-sternberg cells
1. Lymphocyte-predominant
2. Isolated: either cervical or axillary
3. Lymphohistiocytic (popcorn)
33
Staging of lymphoma
1. Stage I
2. Stage II
3. Stage III
4. Stage IV
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
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
1. Follicular lymphoma
2. t(14;18)
3. BCL2 (which prevents apoptosis)
4. Bone marrow
35
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
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
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
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
3 subtypes of diffuse large B cell lymphoma
1. EBV (AIDS/post-transplant)
2. HHV-8 (Kaposis sarcoma)
3. Mediastinal large B cell lymphoma
38
Burkitt lymphoma:
1. Morphology?
2. Translation (3)
3. Age affected
4. Common presentation in US
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
How to differentiate burkitt lymphoma from ALL
Burkitt lymphomas are fast growing and there is a mass present
40
Extranodal marginal zone lymphoma arises in what setting
Autoimmune disorders or chronic infection
41
1. Most common malignant plasma cell dyscrasia?
2. Peak age
3. Presentation?
1. Multiple myeloma
2. 70
3. Multifocal lytic lesions throughout the skeletal system
42
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
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
Lymphoplasmacytic lymphoma:
1. How is it different from CLL/SLL?
2. How is it different from multiple myeloma
1. Many of the tumor cells undergo differentiation to plasma cells
2. No free light chains or lytic bone lesions
44
M component of lymphoplasmacytic lymphoma
IgM - so causes hyperviscosity and worse in cold temperatures
45
Waldenstrom macroglobulinemia - 2 main symptoms
Visual impairment and neurologic problems
46
Common presentation of primary amyloidosis
“Raccoon eyes”, deposits on the tongue, peripheral neuropathy, swollen hands/feet
47
Monoclonal gammopathy of undetermined significance presentation
Asymptomatic - usually found accidentally
48
Myelodysplastic syndromes and chronic myeloproliferative disorders can often transform to __
AML
49
Myelodysplastic syndromes are characterized by __ defects and have a high risk of transforming to __
Maturation; AML
50
2 common abnormalities in myelodysplastic syndromes
Megaloblastoid erythroid precursors (like megaloblastic anemias) and erythroid forms with ring sideroblasts
51
1. Myelodysplastic syndromes have a loss of _ or _
2. Age range
3. Worse prognosis if __ deletion
4. Risk factor
1. 5 or 7
2. 50-70 years old
3. P53
4. Previous chemo
52
What type of mutations are common in chronic myeloproliferative disorders
Tyrosine kinases
53
Polycythemia vera has proliferation of which 3 elements
Erythroid, granulocytic, and megakaryocytic (all myeloid cell line)
54
Relative polycythemia vs reactive polycythemia
Low levels of erythropoietin in polycythemia vera
55
Polycythemia vera:
1. Patient presentation
2. What is in peripheral blood
1. Liver and spleen enlargement, red hue to the skin, itching, bleeding (due to extra platelets)
2. Basophilia
56
Essential thrombocythemia
1. Proliferation of ?
2. Mutation
3. Risk of
1. Platelets (mostly), RBCs and granulocytes
2. JAK2 kinase
3. Risk of thrombosis/bleeding
57
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
1. Marrow fibrosis
2. JAK2
3. Teardrops
4. PDGF and TGF beta
5. Basophilia, hyperuricemia (gout), RBCs with abnormal shapes
58
Langerhans cell histiocytosis
1. Main markers
2. __ in the cytoplasm - what do they look like
3. Mutation
4. Found mostly in the __
1. HLA-DR, CD1a, S100, langerin
2. Birbeck granules - tennis racket shaped
3. BRAF (RAS signaling pathway)
4. Skin
59
Multisystem langerhans cell histiocytosis (Letterer-Siwe disease)
1. Age
2. Symptoms
3. Prognosis
1. < 2 years old
2. Recurrent infections (ear and mastoid), destructive bone lesions, cutaneous lesions, pulmonary lesions
3. Fatal
60
Unisystem langerhans cell histiocytosis (eosinophilic granuloma)
1. Location of lesions
2. Histiocytes mixed with what other 4 cell types
1. Medullary cavities of the bone, skin, lungs, stomach
| 2. Eosinophils, lymphocytes, plasma cells and neutrophils
61
Unisystem langerhans cell histiocytosis (eosinophilic granuloma)
1. Unifocal lesion presentation
2. Multifocal lesion presentation
1. Usually skeletal; asymptomatic
| 2. Children, fever, URIs; hand-schuller-christian disease
62
Hand-schuller-christian disease: 3 symptoms
1. Calvarial bone defects
2. Diabetes insipidus
3. Exophthalmos
