WBC Disorders Flashcards
(111 cards)
1
Q
in the condition seen in the image, there is proliferation of ____ cells, especially ____
____ are markedly increased
A
in the condition seen in the image, there is proliferation of mature myeloid cells, especially granulocytes
basophils are markedly increased
2
Q
in the nodular sclerosis form of the condition seen in the image, there are large ___, surrounded by ____
along with the presence of ____ cells
A
in the nodular sclerosis form of the condition seen in the image, there are large nodules, surrounded by thick fibrous collagen bands
along with the presence of lacunar RS cells cells
3
Q
describe the presentation of the condition seen in the image
A
- presentation:
- pancytopenia, recurrent infections and thrombocytopenia
- extramedullary hematopoiesis = hepatosplenomegaly
- bone pain (due to expansion by the blasts)
4
Q
describe what is seen on urine analysis of the condition seen in the image
A
urine = BJ proteins and kappa & lambda lights chains (AL amyloid)
5
Q
a ____ test/____ antibody test is positive for ____ but negative for ____ in the condition seen in the image
A
a monospot test/heterophile antibody test is positive for EBV but negative for CMV in the condition seen in the image
6
Q
the FAB classification for the condition seen in the image is ____
A
the FAB classification for the condition seen in the image is M3
7
Q
the condition in the image commonly occurs in ____ and is associated with ____ (before or after?) the age of 5
A
the condition in the image commonly occurs in childhood and is associated with Down syndrome after the age of 5
contrast this with AML, where it is associated with Down syndrome before the age of 5
8
Q
what are other causes of marginal zone lymphoma?
A
associated with chronic inflammatory states, like Hashimoto thyroiditis (thyroid gland), Sjogren syndrome (salivary glands) and H. pylori gastritis (stomach)
9
Q
marginal zone lymphoma is an (aggressive or indolent?) lymphoma of small ____ cells
A
marginal zone lymphoma is an indolent lymphoma of small B cells
10
Q
describe the presentation of the condition seen in the image
A
- pruritus (itching) after hot showers b/c basophils make histamine
- extramedullary hematopoiesis → hepatosplenomegaly
- fatigue, weakness, weight loss
11
Q
most translocation in the condition seen in the image confer a better prognosis, except those involving ___
A
most translocation in the condition seen in the image confer a better prognosis, except those involving chr. 11
12
Q
describe the classical vignette for the condition seen in the image
A
55+ year old man presents with back pain
13
Q
in polycythemia vera, due to the JAK2 mutation, the cells are hypersensitive to ____, and there is ____in the serum
A
in polycythemia vera, due to the JAK2 mutation, the cells are hypersensitive to EPO, and there is low EPO in the serum
14
Q
the condition seen in the image is an ____ (aggressive vs. indolent?) ___ cell leukemia
A
the condition seen in the image is an indolent B cell leukemia
15
Q
in the condition seen in the image, upon PB smear, ____ formation is seen
explain this
A
in the condition seen in the image, upon PB smear, rouleaux formation is seen b/c increased serum protein decreases charge between RBCs
16
Q
the treatment of the t(15;17) translocation of the condition seen in the image is with ____
A
the treatment of the t(15;17) translocation of the condition seen in the image is with ATRA which induces differentiation to neutrophils
17
Q
the condition seen in the image most commonly occurs in ____ (age of patients)
A
the condition seen in the image most commonly occurs in older adults >50
18
Q
describe the presentation of the condition in the image
what is NOT seen on presentation?
A
- pancytopenia (normochromic normocytic anemia → fatigue)
- recurrent infections (neutropenia)
- bleeding (thrombocytopenia)
- NO lymphadenopathy
19
Q
the mutation associated with polycythemia vera is ____
A
the mutation associated with polycythemia vera is JAK2 mutation
20
Q
on lymph node biopsy of the condition seen in the image, a ____ appearance is seen with microscopy
since the cell are rapidly dividing, ____ figures are also seen and stains positive with ____
A
on lymph node biopsy, a starry sky appearance is seen with microscopy (high mitotic rate + apoptosis + macrophages)
since the cell are rapidly dividing, mitotic figures are also seen and stains positive with Ki67
21
Q
describe what is seen on BM biopsy in myelodysplastic syndrome (MDS)
A
usually hypercellular, but ineffective disorganized hematopoiesis
22
Q
the translocation associated with good prognosis of the condition seen in the image is ____
A
the translocation associated with good prognosis of the condition seen in the image is t(12,21)
23
Q
explain how the word “TRAP” helps determine clinical features of the condition seen in the image
A
TRAP = tartrate-resistant acid phosphatase staining of hairy cell leukemia
cells get trapped in red pulp = splenomegaly
cells get trapped in bone marrow = dry aspiration
cells trapped in other locations = NO lymphadenopathy (where it should normally be)
24
Q
severe neutropenia is most commonly caused by ___
A
severe neutropenia is most commonly caused by drugs
- predictable, dose-related (e.g. chemotherapy)
- idiosyncratic; many drugs, usually immune-mediated by suppression of marrow precursors
25
in the B-cell form of the condition seen in the image, the associated CD markers are ____ (3), and is also ____ positive
classic B cell CD markers = **CD10, 19, 20**
## Footnote
**also TdT positive**
26
since the virus causing the condition in the image remains dormant in B-cells, a complication is \_\_\_\_\_
since the virus causing the condition in the image remains dormant in B-cells, a complication is **non-Hodgkin B cell lymphoma (Burkitt's lymphoma)**
27
in the condition seen in the image, there is an increase in neutrophils, so why are there recurrent infections in these patients?
PMNs are **monoclonal with decreased LAP** (leukocyte alkaline phosphatase) score, and are therefore unable to fight off infections
28
on flow cytometry of the condition seen in the image, the cells are CD\_\_\_\_ positive (3 CD markers)
on flow cytometry of the condition seen in the image, the cells are **CD10, CD19, CD20 (B cells!)** positive
29
describe what is seen on serum protein electrophoresis of the condition seen in the image
**M spike** (monoclonal Igs) = **IgG and IgA**
30
the translocation associated with a poor prognosis of the condition seen in the image is \_\_\_\_
the translocation associated with a poor prognosis of the condition seen in the image is **t(9,22)**
31
15% of the condition seen in the image are precursor T-cell neoplasms that usually present as a _____ mass in an adolescent male
15% of the condition seen in the image are precursor T-cell neoplasms that usually present as **a mediastinal (thymic) mass** in an adolescent male
32
about 40% of follicular lymphomas transform to a more aggressive lymphoma, usually \_\_\_\_
about 40% of follicular lymphomas transform to a more aggressive lymphoma, usually **diffuse large B-cell lymphoma (DLBL)**
33
the most common cause of death in the condition seen in the image is \_\_\_\_
why?
the most common cause of death in the condition seen in the image is **recurrent infections**
although there is an increase in Igs, there is **monoclonal proliferation and therefore no antigenic diversity**
34
describe the presentation of the condition seen in the image
* painless rubbery enlargement of one or more lymph nodes (cervical & axillary)
* **pain only after drinking alcohol**
* B symptoms:
* fever
* night sweats
* weight loss
35
a complication of the condition in the image is transformation to \_\_\_\_
a complication of the condition in the image is transformation to **AML**
36
the T cell form of the condition seen in the image is associated with CD markers ___ and is also ____ positive
the T cell form of the condition seen in the image is associated with CD markers **CD2 - CD8**
and is also **TdT positive**
37
the condition seen in the image can also transform into \_\_\_\_, which is also called ____ transformation
the condition seen in the image can also transform into **DLBL**, which is also called **Richter's transformation**
38
list causes of eosinophilic leukocytosis
* allergic disorders
* skin diseases
* bullous pemphigus & pemphigoid, dermatitis herpetiformis
* parasitic infestations
* drug reactions
* malignancies
* Hodgkin's, T-cell
* collagen vascular diseases, vasculitis
39
list the diseases that can transform to AML
* myeloproliferative disorders (blast crisis)
* CML = most common
* MDS
* essential thrombocytosis
* polycythemia vera
* myelofibrosis
* CLL
* PNH (can also transform to MDS)
40
a complication of the condition seen in the image is ___ rupture which can lead to \_\_\_\_
therefore, patients with this condition should avoid \_\_\_\_
a complication of the condition seen in the image is **splenic** rupture which can lead to **peritonitis → hypovolemic shock**
therefore, patients with this condition should avoid **contact sports**
41
based on the condition seen in the image, describe the t(15;17) translocation
* translocation of the **retinoic acid receptor (RAR) on** **chr****. 17**with the**PML gene on****chr****. 15**
* the PML/RAR fusion protein blocks myeloid differentiation at the **promyelocytic stage** (probably by inhibiting the function of normal retinoic acid receptors)
42
in follicular lymphoma, the tumor arises from \_\_\_\_
list the 2 patterns of growth, 2 types of cells, and their behavior
in follicular lymphoma, the tumor arises from **germinal center B cells**
2 types = nodular and diffuse
2 cells = small and large
**diffuse and large = most aggressive**
43
describe what is seen on lymph node biopsy of the condition seen in the image
* LN: diffuse replacement by predominantly small round lymphocytes with few larger cells often concentrated in pale **"proliferation centers"**
44
the FAB classification for the monocytic variant of the condition seen in the image is \_\_\_\_
the FAB classification for the monocytic variant of the condition seen in the image is **M5**
45
acute leukemias normally have no \_\_\_\_
while
chronic leukemias do have ____ (with ____ being the exception)
acute leukemias normally have no **lymphadenopathy**
while
chronic leukemias do have **lymphadenopathy** (with **hairy cell leukemia** being the exception)
46
in the condition seen in the image, the pathogenesis is mature, non-functional malignant ____ that coexpress CD\_\_\_ and CD\_\_\_
in the condition seen in the image, the pathogenesis is naive, non-functional malignant **B-cells** that coexpress **CD20** and **CD5**
47
on BM aspiration in the condition in the image, what is seen?
**DRY TAP due to massive fibrosis**
48
in the condition seen in the image, deletions/monosomy of chr. ___ usually occur in \_\_\_\_
in the condition seen in the image, deletions/monosomy of chr . **5/7** usually occur in **older adults with AML, myelodysplasia (MDS), post-chemo/radiotherapy**
49
in the condition seen in the image, there is a mutation in ____ which leads to ____ secreting \_\_\_\_
in the condition seen in the image, there is a mutation in **JAK2** which leads to **atypical megakaryocytes** secreting **TGF-B & PDGF** → abnormal deposition of collagen → BM fibrosis → pancytopenia
50
list causes of neutrophilic leukocytosis (neutrophilia)
* infections
* pyogenic bacteria
* sterile inflammation
* acute inflammation
* gout, RA
* acute hemorrhage
* malignancy
51
in the condition seen in the image, a complication is ___ and ___ failure due to an increase in \_\_\_\_
in the condition seen in the image, a complication is **gout** and **renal failure** due to an increase in **uric acid (hyperuricemia)**
52
in the condition seen in the image, there is massive ____ hematopoiesis which leads to \_\_\_\_
in the condition seen in the image, there is massive **extramedullary** hematopoiesis which leads to **hepatosplenomegaly**
53
the most common cause of death in the condition seen in the image is ___ due to a decrease in ____ score
the most common cause of death in the condition seen in the image is **recurrent infxns** due to a decrease in **LAP** score
54
what is the most common cause of death of the condition seen in the image?
most common COD = recurrent infections due to neutropenia & hypogammaglobulinemia
55
describe what is seen on PB smear of the condition in the image, and what it is stained with
* PB smear: **malignant B cells with cytoplasmic fine hair-like projections**
* **TRAP stain positive** (TRAP = tartrate-resistant acid phosphatase)
56
describe what is seen in the PB smear in the condition in the image
**teardrop cells (dacrocytes) & leukoerythroblastosis (immature RBCs and WBCs)**
57
describe a characteristic finding that is seen on PB smear of the condition seen in the image
PB smear: **smudge cells** (mature B cells that get crushed due to fragile membranes)
58
on bone marrow biopsy of the condition seen in the image, there are atypical, reactive, ____ aka \_\_\_\_
on bone marrow biopsy of the condition seen in the image, there are **atypical, reactive, CD8 T cells aka Downey cells**
59
what are the characteristic CD markers in the condition seen in the image?
**CD20, *_CD11c_*, CD25, *_CD103_***
60
describe the presentation of the t(15;17) translocation in the condition seen in the image
* presentation: pancytopenia, recurrent infxns, thrombocytopenia
* extramedullary hematopoeisis: hepatosplenomegaly
* **Auer rods are thrombogenic → DIC**
* Waterhouse Friderichsen syndrome
* destruction of adrenal cortex **→ Addison's disease**
61
the condition seen in the image is ____ lymphoma of ___ cells caused by \_\_\_\_
what translocation is associated with this condition?
the condition seen in the image is **non-Hodgkin lymphoma** of **medium B cells** caused by **EBV**
## Footnote
**t(8, 14): c-myc & heavy chain of Ig**
62
describe the image
**follicular lymphoma**
nodular aggregates of lymphoma cells are present throughout lymph node
63
the mutation in the T-cell form of the condition seen in the image is usually in the ____ (gene)
the mutation in this condition is usually in the **NOTCH-1 (point mutation)** gene
up to 70% of T-ALLs have gain-of-function mutations in NOTCH1, a gene that is essential for T cell differentiation
64
list the 4 causes of renal failure in the condition seen in the image
1. AL amyloidosis
2. increased uric acid = toxic to renal tubules & uric acid stones
3. increased Ca2+ = toxic and makes stones
4. recurrent kidney infxns = acute pyelonephritis
65
list causes of reactive monocytosis
* chronic infxns
* TB, rickettsiosis, bacterial endocarditis, malaria
* collagen vascular diseases
* SLE
* IBD
* UC
66
list poor prognostic factors for the condition seen in the image
poor prognosis = **B symptoms** (fever, weight loss, night sweats), **extranodal spread**
67
for predictable neutropenia, such as following myelosuppressive chemotherapy, treatment includes \_\_\_\_
for predictable neutropenia, such as following myelosuppressive chemotherapy, treatment includes **G-CSF (drug = filgrastim)**
68
\_\_\_\_ cells are commonly seen in a PB smear of the condition seen in the image
**tear-drop** cells are commonly seen in a PB smear of the condition seen in the image
69
describe the image
**follicular lymphoma**
follicular lymphoma cells show strong BCL2 staining
70
describe the nodular sclerosis type of the condition seen in the image
* commonest subtype of HL
* mediastinal involvement very common
* large nodules, at least partly surrounded by thick fibrous collagen bands
* presence of a type of RS cell called **lacunar RS cells**
71
the best treatment for the condition seen in the image is with \_\_\_\_
treatment for the condition seen in the image is with **imatinib/Gleevec/STI571 = TK inhibitor**
72
describe the image
neoplastic Reed-Sternberg cells (altered germinal center B-lymphocytes) in the minority
non-neoplastic inflammatory cells (lymphocytes, plasma cells, eosinophils) in the background in the majority
73
describe the etiology of the condition seen in the image
**EBV (mostly)** and CMV
"kissing disease" that is common in HS kids
74
the African (aka endemic) variant of the condition seen in the image occurs _____ (location)
whereas
the sporadic (non-endemic) variant occurs \_\_\_\_\_
the African (aka endemic) variant of the condition seen in the image occurs **on the mandible**
whereas
the sporadic (non-endemic) variant occurs **in the abdomen (ileocecal)**
75
describe the pathogenesis of bone symptoms seen in the condition in the image
* myeloma-derived factors upregulate the expression of RANKL, which in turn activate osteoclasts → produces punched-out lytic lesions in bone
* most commonly affected = lumbar spine = back pain
* breaking bone = hypercalcemia
76
the condition seen in the image is composed of ____ cells which are altered \_\_\_\_\_
the condition seen in the image is composed of **Reed-Sternberg** cells which are altered **germinal center B-lymphocytes**
77
the most common site of a marginal zone lymphoma is the ____ (superimposed on \_\_\_\_)
the most common site of a marginal zone lymphoma is the **stomach** (superimposed on **Helicobacter gastritis**)
antibiotic treatment of Helicobacter gastritis has caused regression of some early stomach marginal zone lymphomas
78
describe the presentation of the condition in the image
a rash forms AFTER administration of \_\_\_\_
* fever
* sore throat
* lymphadenopathy (cervical, axillary, inguinal)
* hepatosplenomegaly
a rash forms AFTER administration of **ampicllin**
79
in the condition seen in the image, ___ is the most important prognostic indicator
what factors cause poor prognosis?
in the condition seen in the image, **stage** is the most important prognostic indicator
* poor prognosis = increased RS cells, B symptoms, extranodal spread
80
describe the image
**Burkitt's lymphoma - lymph node**
The tumor cells and their nuclei are fairly uniform, giving a monotonous appearance
Note the high level of mitotic activity (arrowheads) and prominent nucleoli
The "starry sky" pattern is produced by interspersed, lightly staining, normal macrophages
81
in the condition seen in the image, a complication is transformation to \_\_\_
in the condition seen in the image, a complication is transformation to **AML which can ultimately cause DIC**
82
on LN biopsy of the condition seen in the image, the causative cells have a ____ nucleus
on LN biopsy, RS cells have a **mirror-imaged Owl's eye nucleus**
83
the most common cause of death in the condition seen in the image is \_\_\_\_
the most common cause of death in the condition seen in the image is **recurrent infections**
84
chromosomal translocations of the condition seen in the image usually occur in \_\_\_\_
while
deletions/monosomy of chr. 5 & 7 usually occur in \_\_\_\_
chromosomal translocations of the condition seen in the image usually occur in **younger adults with de novo AML**
e.g. t(15,17), t(8,21), t(16,16)
85
describe bone marrow biopsy of the condition seen in the image
* BM: **dry tap due to fibrosis** (TGF-B and PDGF)
* infiltrate of small lymphocytes with abundant pale cytoplasm ("fried egg" appearance) enmeshed in reticulin
* usually fibrosis means marrow cannot be aspirated
86
describe what is seen on PB smear in the condition in the image
striking **left-shifted leukocytosis, with mostly neutrophils & precursors (metamyelocytes, myelocytes)**
## Footnote
**increase in eosinophils and basophils**
87
describe what is seen on BM biopsy in the condition in the image
* BM biopsy: **collagen stains black with reticulin b/c BM is hypocellular**
88
the classical form of the cells seen in the condition express CD\_\_\_ and CD\_\_\_
while
the variant form of the cells seen in the condition express CD\_\_\_ and CD\_\_\_\_
the classical form of the cells seen in the condition express **CD15** and **CD30**
while
the variant form of the cells seen in the condition express **CD20** and **CD45**
89
the translocation associated with the condition in the image is t(\_\_\_), which results in \_\_\_\_
the translocation associated with the condition in the image is t(**9;22**), which results in **overactivity of TK receptor**
90
describe the T-cell form of the condition seen in the image
91
in the condition seen in the image, there is extramedullary hematopoiesis, which leads to _____ that has a _____ appearance
in the condition seen in the image, there is extramedullary hematopoiesis, which leads to **splenomegaly** that has a **beefy red** appearance **(accumulation of hairy cells in red pulp)**
92
describe what is seen in the image
**Hodgkin lymphoma, nodular sclerosis type**
well-defined bands of pink, acellular collagen that subdivides the tumor into nodules
93
describe how to differentiate between ALL and AML
94
what is usually the first symptom seen in the condition in the image?
sometimes the first symptom is a **dragging sensation in the abdomen caused by splenomegaly**
95
describe what is seen in the image
**Hodgkin lymphoma**
Lacunar RS cells
96
the M4/M5 variant of the condition seen in the image characteristically involves ____ infiltrating the \_\_\_\_
the **M4/M****5 (aka acute monocytic leukemia)**variant of the condition seen in the image characteristically involves**blasts**infiltrating the**gums**
97
in the condition seen in the image, there are ____ present in the cell, which are thrombogenic and can ultimately lead to \_\_\_\_
in the condition seen in the image, there are **Auer rods** present in the cell, which are thrombogenic and can ultimately lead to **DIC**
98
the pathogenesis of the condition in the image is that the virus infects ____ cells but ____ cells are the reactive ones
the pathogenesis of the condition in the image is that the virus infects **B cells** but **T cells** are the reactive ones
99
describe the presentation of the condition seen in the image
* **pancytopenia** = malignant B cells activate normal B lymphocytes to form IgG → immune-mediated damage to RBCs (warm autoimmune hemolytic anemia) → **(+) Coombs and spherocytes**
* **recurrent infections** caused by **neutropenia** and **hypogammaglobulinemia**
100
the M4/M5 variant of the condition in the image involves proliferation of ___ and the subtype lacks \_\_\_
the M4/M5 variant of the condition in the image involves proliferation of **monoblasts** and the subtype lacks **MPO**
101
the most common cause of death in the condition seen in the image is \_\_\_\_
the most common cause of death in the condition seen in the image is **recurrent infxns**
102
the condition seen in the image is positive for ____ (which helps differentiate it from ___ which is positive for \_\_\_\_)
the condition seen in the image is positive for **MPO** (which helps differentiate it from **ALL** which is positive for **TdT**)
103
describe what is seen in the image
104
in follicular lymphoma, the translocation occurs between \_\_\_\_
in follicular lymphoma, the translocation occurs between **t(14,18), between the heavy chain of Ig and BCL2**
105
the M7 variant of the condition seen in the image (aka \_\_\_\_) lacks ____ and is associated with \_\_\_\_
the M7 variant of the condition seen in the image (aka **acute megakaryoblastic leukemia**) lacks **MPO** and is associated with **Down sydrome *before* the age of 5**
"need to karyo child under 5"
106
on lymph node biopsy of the condition seen in the image, there is \_\_\_\_\_
on lymph node biopsy of the condition seen in the image, there is **paracortical hyperplasia (T cells)**
107
list causes of reactive lymphocytosis
* viral infxns
* EBV, Hep A, CMV
* B. pertussis infxn
108
list other potential treatments for the condition seen in the image
* **allogenic BM transplantation**
* **interferon-a** slows down disease
* **hydroxyurea** = "gentle" chemotherapy
109
describe the 3 types of the condition seen in the image
* African/endemic: 100% EBV; doubling time = 24 hours
* painless mass in mandible → mandibular lymphadenopathy
* American/sporadic
* mesenteric lymph nodes → intestinal obstruction (ileocecal)
* HIV associated BL
110
the condition seen in the image has a good response to ____ and most ____ can be cured, but ____ do worse
the condition seen in the image has a good response to **high dose chemotherapy** and most **kids/young adults** can be cured, but **older adults** do worse
111
describe the pathogenesis of the condition seen in the image
**monoclonal proliferation of plasma cells in the bone marrow**
