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WBC disorders Flashcards

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1
Q

Acute Promyelocytic Leukemia (APL) is characterized by

A

t(15;17) of Retinoic Acid Receptor (RAR)

RAR disruption blocks maturation and promyelocytes accumulate

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2
Q

Adult T-cell leukemia/lymphoma clinical features

A
  1. Rash
  2. Generalized lymphadenopathy with hepatosplenomegaly
  3. Lytic bone lesions with hypercalcemia
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3
Q

ALL is

A

neoplastic accumulation of lymphobasts in the BM

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4
Q

ALL most commonly arises in

A

Children with Down Syndrome

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5
Q

APL can increase the risk for

A

DIC

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6
Q

B-ALL characterized by

A
  1. TdT
  2. CD10
  3. CD19
  4. CD20
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7
Q

Basophilia seen in

A

Chronic Myeloid Leukemia

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8
Q

Blasts are

A

Large immature cells often with punched out nucleoli

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9
Q

Causes of Lymphopenia

A
  1. Immunodeficiency
  2. High cortisol state induces apoptosis of lymphocytes
  3. Autoimmune destruction
  4. Whole body radiation
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10
Q

CD8 T-cell response to Infections mononucleosis leads to

A
  1. Generalized lymphadenopathy due to T-cell hyperplasia
  2. Splenomegaly due to T-cell hyperplasia in the PALS
  3. High WBC count with atypical lymphocytes
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11
Q

Clinical features of Hairy Cell Leukemia

A
  1. Splenomegaly: accumulates in red pulp
  2. Dry tap on BM aspiration due to marrow fibrosis
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12
Q

CLL complications include

A
  1. Hypogammaglobulinemia: most common cause of death
  2. IgM autoimmune hemolytic anemia
  3. May transform to diffuse large B-cell lymphoma (Richter transformation)
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13
Q

CLL naive B cells co-express

A

CD5 and CD20

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14
Q

CML is

A

Neoplastic proliferation of mature myeloid cells, especially granulocytes and their precursors

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15
Q

CML translocation is

A

t(9;22) philadelphia chromosome generates BCR-ABL fusion that increases tyrosine kinase activity

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16
Q

Complications of Myeloproliferative disorders (MPD) are

A
  1. Increased risk of hyperuricemia and gout due to high turnover of cells
  2. Progression to Marrow fibrosis or transformation to acute leukemia
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17
Q

EBV primarily infects

A
  1. Oropharynx: pharyngitis
  2. Liver: hepatits and hepatomegaly with elevated liver enzymes
  3. B cells
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18
Q

Eosinophilic granuloma is

A

Benign proliferation of Langerhans cell in bone

No skin involvement

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19
Q

Essential thrombocytothemia is

A

Neoplastic proliferation of mature myeloid cells especially plateletsb

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20
Q

ET is associated with what mutation

A

JAK2 kinase

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21
Q

First line treatment of CML is

A

Imantinib

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22
Q

Follicular hyperplasia of lymph node seen in

A

RA

Early signs of HIV

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23
Q

Follicular lymphoma can progress to

A

diffuse large B-cell lymphoma

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24
Q

Follicular lymphoma is

A

Neoplastic proliferation of small B cells (CD20+) that form follicle-like nodules

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25
Follicular lymphoma treatment
Only for patients who are symptomatic Chemotherapy or **rituximab**
26
Hairy cell leukemia cells are positive for
TRAP
27
Hairy cell leukemia is
Neoplastic proliferation of mature B cells characterized by **Hairy cytoplasmic processes**
28
Hand-Schüller-Christian disease
Malignant Langerhans cells
29
Hand-Schüller-Christian disease presentation
1. Scalp rash 2. Lytic skull defects 3. DI 4. Exophthalmos in children
30
Immature cells are characterized by
Decreased Fc receptors (**CD16**)
31
In what disorder do blasts infertrate gums
Acute monocytic leukemia
32
Infectious mononucleosis is caused by
EBV infection that results in Lymphocytic lekocytosis
33
Lacunar cells are seen in what type of Hodgkins lymphoma
Nodular sclerosis
34
Letterer-Siwe disease is
Malignant proliferation of langerhans cells Skin rash with cystic skeletal defects
35
Leukocytosis is
Increase WBC
36
Leukopenia is
Decreased WBC
37
Lymphoblasts in ALL are characterized by
Positive nuclear staining for **TdT**
38
Lymphopenia refers to
A decreased number of circulating lymphocytes
39
M spike in Multiple myeloma due to
Monoclonal IgG or IgA
40
Mantel cell lymphoma translocation
t(11;14) causing an overexpression of **cylcin D1** promotes **G1/S transition**
41
Marginal zone lymphoma associated with
1. Hashimoto 2. Sjögren 3. H pylori gastritis
42
Mediastinal (thymic) mass in teenagers
T-ALL
43
Multiple myeloma is
Malignant proliferation of plasma cells in the BM High serum **IL-6**
44
Mycosis fungoides is
neoplastic proliferation of mature CD4 T cells that infiltrate the skin producing localized skin rash, plaques, and nodules
45
Myeloblasts are characterized by
Positive cytoplasmic staining for MPO MPO crystal aggregates may be seen as **Auer Rods**
46
Neutropenia is
Decreased number of circulating PMN
47
Non-Hodgkins lymphoma include
1. Small B cell 1. Follicular 2. Mantel cell 3. Marginal zone 4. Small lymphocytic 2. Burkitt lymphoma 3. Diffuse large B-cell lymphoma
48
Pautrier microabscesses are
aggregates of neoplastic cells in the epidermis seen in Mycosis fungoides
49
Polycythemia vera is associated with what mutaiton
JAK2 kinase mutation
50
PV clinical symptoms
1. Blurry vision and headache 2. Increase risk of Venous thrombosis 3. Flushed face due to congestion 4. Itching, especailly after bathing
51
Reed-Sterberg cells are
1. Malignant cells seen in Hodgkin lymphoma 2. Large B cels with multilobbed nuclei with prominent nucleoli (**'owl-eyed nuclei'**) 3. Positive for **CD15 and CD3-**
52
Rouleaux formation of RBC is
Increases serum protein decreases charge between RBCs attaching them together seen in Multiple Myeloma
53
Sezary cells are
Lymphocytes with cerebriform nuclei seen on blood smear of Mycosis Fungoides
54
T-ALL characterized by
TdT CD2 to CD8
55
Waldenström Macroglobulinemia is
B-cell lymphoma with monoclonal IgM production
56
What causes Neutropenia
1. Drug toxicity damages stem cells 2. Severe infection from G(-) causing an incresed movement of PMN to tissue
57
What is follicular lymphoma translocation
t(14;18) leading to overexpression of BCL2
58
What is seen on CLL blood smear
Increased lympohcytes and smudge cells
59
What is the treatment of APL
All-*trans*-retinoic acid
60
What is used to treat Hairy cell leukemia
1. 2-CDA (cladribine) 2. Adenosine deaminase inhibitor
61
What translocation of B-ALL has bad prognosis
t(9;22)
62
What translocation of B-ALL has good prognosis
t(12;21)

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