Hematology WBC disorders Flashcards
(86 cards)
1
Q
CD34+
A
Hematopoetic Stem Cells
2
Q
GM-CSF & G-CSF treat?
A
Neutropenia
3
Q
Decreased CD16
A
CD16= Fc rec
Immature Neutrophils
4
Q
WBC increased by Type I hypersensitivity, Hodgkin’s lymphoma
A
Eosinophils
5
Q
WBC increased by CML
A
Basophils
6
Q
WBC increased by Bordatella pertussis
A
Lymphocytes
Can’t leave circulation
7
Q
CD8+ Tcell leukocytosis
A
EBV
sometimes CMV
8
Q
TdT+ stain
Down Syndrome after 5
A
ALL
Acute Lymphoblastic Leukemia
9
Q
CD10, CD19, CD20
A
B-ALL
MC vs. T-ALL
TdT+
10
Q
t(12:21)
A
B-ALL
MC in kids, good prognosis
t(9:22) philadelphia
TdT+
11
Q
CD2-CD8
Negative CD10
A
T-ALL
TdT+
12
Q
MPO+
A
Acute Myeloid Leukemia AML
Auer rods
13
Q
t(15:17)
A
Acute Promyelotic Leukemia
RAR (17) -> blocks maturation -> promyelocyte^ -> DIC risk^
14
Q
Infiltrate gums
A
Acute Monocytic leukemia
Lacks MPO
AML subtype
15
Q
Down Syndrome before age 5
A
Acute megakaryoblastic leukemia
Lack MPO
AML subtype
16
Q
CD5 and CD20
MC leukemia overall
A
Chronic Lymphocytic Leukemia Smudge cells Hypogammaglobulinemia Infection MCC of death Autoimmune hemolytic anemia Transformation (Richter) to Diffuse Large B-cell Lymphoma
17
Q
TRAP+
A
Hairy Cell Leukemia
Lacks LAD
18
Q
Splenomegaly (Red Pulp)
A
Hairy Cell Leukemia
Dry tap on BM aspiration due to fibrosis.
Lacks LAD
19
Q
Responds to 2-CDA
A
Hairy Cell Leukemia
(cladribine) Adenosine deaminase inhibitor - causes adenosine to accumulate and becomes toxic in neoplastic B-cells
20
Q
Associated with HTLV-1
A
Adult T-Cell Leukemia/lymphoma (ATLL)
MC seen in Japan and Caribbean
21
Q
Rash, Generalized LAD with Hepatosplemnomegaly, and lytic (punched-out) bone lesions with hypercalcemia
A
Adult T-Cell Leukemia/Lymphoma (ATLL)
22
Q
Neoplastic proliferation of mature CD4+ T cells
A
Adult T-Cell Leukemia/Lymphoma (ATLL)
Mycosis Fungoides
23
Q
Skin rash, plaques, and nodules created by neoplastic cells
-Pautrier Microabscesses
A
Mycosis Fungoides
Neoplastic proliferation of mature CD4+ T cells that infiltrate the skin
24
Q
Sezary syndrome
A
Mycosis Fungoides
Cells spread to involve blood.
Characteristic lymphoctytes with cerebriform nuclei on blood smear
25
Neoplastic proliferation with average age of 50-60 years
Myeloproliferative disorders
| CML, PV, ET, Myelofibrosis
26
Hypercellular BM
Increased risk for hyperuricemia and gout
Progression to BM fibrosis or transformation to acute leukemia
Myeloproliferative disorders
(CML, PV, ET, Myelofibrosis)
Classified based on dominant myeloid cell produced
Hyperuricemia and gout due to high turnover of cells
27
Characteristic Basophilia
Chronic Myeloid Leukemia (CML)
| Increased mature myeloid cells
28
t(9:22)
Chronic Myeloid Leukemia (CML)
Philadelphia chromosome -> BCR-ABL fusion -> ^tyrosine kinase activity
LAP-
29
Imatinib treatment
Chronic Myeloid Leukemia (CML)
Blocks tyrosine kinase activity
Enlarging Spleen suggests progression to AML (2/3) or ALL (1/3)
LAP-
30
JAK2 kinase mutation
```
Polycythemia Vera (PV)
Essential Thrombocythemia (ET)
Myelofibrosis - 50%
```
31
Blurry Vision and HA
Risk of venous thrombosis
Flushed face (plethora)
Itching post bath
Polycythemia Vera (PV)
Venous thrombosis in hepatic vein, portal vein, dural sinus
Itching due to histamine release from mast cells
EPO decreased
32
Hydroxyurea treatment
Polycythemia Vera (PV)
1st line tx Phlebotomy
2nd line tx Hydroxyurea
EPO decreased in PV
33
Increased risk of bleeding and/or thrombosis
| JAK2 mutation
Essential thrombocythemia (ET)
Rarely progress to BM fibrosis or acute leukemia
No significant risk of hyperuricemia or gout
34
Excess PDGF
Myelofibrosis
causes BM Fibrosis
JAK2 mutation
35
Splenomegaly
Leukoerythroblastic smear
Increased risk of infection, thrombosis, and bleeding
Myelofibrosis
1. Splenomegaly due to extramedullary hematopoiesis
2. Leukoerythroblastic smear (tear-drop RBCs, nucleated RBCs, and immature granulocytes
JAK2 mutation
36
Painless LAD
```
Chronic inflammation (chronic lymphadenitis)
Metastatic carcinoma
Lymphoma
```
37
Follicular hyperplasia
B-cell region seen with RA and early stages of HIV infection
38
Paracortex hyperplasia
T-cell region seen with viral infection (Infectious mononucleosis)
39
Hyperplasia of sinus histiocytes
Seen in LN that are draining a tissue with CA
40
Small B-cell lymphomas
Follicular lymphoma
Mantle cell lymphoma
Marginal zone lymphoma
Small Lymphocytic lymphoma (CLL that involves tissues)
CD20+
41
Intermediate-sized B cells lymphoma
Burkitt lymphoma
42
Large B cells lymphoma
Diffuse large B-cell lymphoma
43
CD20+ that form follicle-like nodules
```
Follicular Lymphoma (NHL)
Presents in late adulthood with painless LAD
```
44
t(14:18)
Follicular Lymphoma (NHL)
BCL2 on 18 to
IG heavy chain on 14
BCL2 inhibits apoptosis
45
Rituximab treatment for symptomatic patients
```
Follicular Lymphoma (NHL)
anti-CD20 antibody, low dose
```
46
Follicular Lymphoma with enlarging Lymph Node
Risk for progressing to Diffuse Large B-cell Lymphoma
47
Follicular Lymphoma characteristics vs Reactive follicular hyperplasia
1. Disruption of normal lymph node architecture
2. Lack of tingible body macrophages in germinal centers
3. Bcl2 espression in follicles
4. Monoclonality
48
t(11:14)
Mantle Cell Lymphoma
Late adulthood with painless LAD
CyclinD1 on 11 to
Ig heavy chain on 14
49
cyclin D1
Mantle Cell Lymphoma
t(11:14)
Promotes G1/S transition in the cell cycle
50
Lymphoma associated with Chronic inflammatory states like Hashimoto thyroiditis, Sjogren syndrome, and H. pylori gastritis
Marginal Zone Lymphoma
Marginal zone is formed by post-germinal center B cells (CD20+)
MALToma in mucosal sites
- gastric MALToma may regress with tx of H. pylori
51
Lymphoma associated with EBV
| Extranodal mass in child or young adult
Burkitt Lymphoma
African - lower jaw
Sporadic - abdomen
52
t(8:14)
Burkitt Lymphoma
Translocation of c-myc on 8
Ig Heavy chain on 14
c-myc promotes cell growth
53
Starry-sky
Burkitt Lymphoma
Characterized by high mitotic index
54
Large B cells (CD20+) that grow diffusely in sheets
Diffuse Large B-Cell Lymphoma
| Clinically aggressive
55
MC form of NHL
Diffuse Large B-Cell Lymphoma
| Clinically aggressive
56
Late adulthood as enlarging LN or extranodal mass
| Sporadic formation or transformation of low grade lymphoma
Diffuse Large B-Cell Lymphoma
57
CD15 and CD30+
Reed-Sternberg cells
| Hodgkin lymphoma
58
Large B cells with multilobed nuclei and prominent nucleoli
Reed-Sternberg cells
Hodgkin lymphoma
'owl-eye nucleoli'
59
Symptoms of Reed-Sternberg cell's secretion of cytokines
1. Occasionally results in 'B' sx (F/C, weight loss, night sweats)
2. Attract reactive lymphocytes, plasma cells, macrophages, and eosinophils
3. May lead to fibrosis
60
Hodgkin Lymphoma's subtypes
1. Nodular sclerosis
2. Lymphocyte-rich
3. Mixed cellularity
4. Lymphocyte-depleted
61
MC subtype of HL
Nodular sclerosis
62
Presents as enlarging cervical or mediastinal lymph node in a young adult, usually female
Nodular sclerosis (HL)
63
Lymph node is divided by bands of sclerosis
| Lacunar cells
```
Nodular sclerosis (HL)
Lacunar cells - Reed-sternberg cells present in lake-like spaces
```
64
HL subtype with best prognosis
Lymphocyte-rich
65
HL subtype associated with abundant eosinophils
Mixed cellularity (RS cells produce IL-5)
66
HL subtype that is the most aggressive; seen in elderly and HIV+ individuals
Lymphocyte-depleted
| Seen in elderly and HIV+ individuals
67
MC primary malignancy of bone
Multiple myeloma
68
MC malignant lesions of bone
Metastatic cancer
69
High serum IL-6 may be present
Multiple myeloma
| IL-6 stimulates plasma cell growth and Ig production
70
Bone pain with hypercalcemia
| Lytic lesions on x-ray
Multiple myeloma
Cells activate the RANK rec on osteoclasts.
Lytic lesions in vertebrae and skull
Increased risk of fracture
71
Elevated serum protein
Multiple myeloma
Cells produce Ig
M spike on SPEP
MC due to monoclonal IgG or IgA
72
Increased risk of infection - Monoclonal Ab lacks diversity
Multiple myeloma
73
MC cause of death in Multiple myeloma
Infection
74
Rouleaux formation of RBCs on Blood smear
Multiple myeloma
| Increased serum protein decreases charge b/w RBCs
75
Primary AL amyloidosis
Multiple myeloma
| Free light chains circulate in serum and deposit in tissues
76
Proteinuria - Bence Jones protein
Multiple myeloma
Bence Jones - Free light chains excreted in Urine
Deposition in kidney tubules leads to risk for RF (myeloma kidney)
77
Increased serum protein with M-spike on SPEP, Common in elderly
w/o sx
Monoclonal Gammopathy of Undetermined Significance (MGUS)
No lytic bone lesions, Hypercalcemia, AL amyloidosis, Bence jones
5% of 70 y.o.
1% pts develop Multiple myeloma yearly
78
B-cell lymphoma with monoclonal IgM production
Waldenstrom Macroglobulinemia
79
Generalized LAD; w/o lytic bone lesions
Increased serum protein with M-spike on SPEP
Visual and neurologic deficits (retinal hemorrhage or stroke)
Bleeding
Waldenstrom Macroglobulinemia
IgM - causes hyperviscosity
Viscous serum - defective platelet aggregation
80
Treated with plasmapheresis in acute complication
Waldenstrom Macroglobulinemia
| Removes IgM from the serum
81
CD1a+ and S100+ by immunochemistry
Langerhans cell Histiocytosis
82
Birbeck granules
Langerhans cell Histiocytosis
| tennis racket appearing
83
Malignant proliferation of Langerhans cells
Letterer-Siwe Disease
| Hand-Schuller-Christian Disease
84
Skin rash and cystic skeletal defects in infant (
Letterer-Siwe Disease
Multiple organs may be involved; rapidly fatal
Malignant Langerhans cells
85
Scalp Rash, lytic skull defects, diabetes insipidus, and exophthalmos in a child
Hand-Schuller-Christian Disease
Malignant Langerhans cells
86
Pathologic fracture in an adolescent
Skin not involved
Biopsy?
Eosinophilic Granuloma
Benign Langerhans cells in bone
Biopsy: Langerhans cells w/ mixed inflammatory cells, including eosinophils
