Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

HEMATOLOGY 1 > WBC ANOMALIES > Flashcards

WBC ANOMALIES Flashcards

(83 cards)

Start Studying
1
Q

Niemann-Pick Disease (NPD) is caused by a deficiency in what enzyme?

A

Sphingomyelinase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Niemann-Pick Disease (NPD) inheritance pattern?

A

Autosomal recessive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Niemann-Pick Disease (NPD) is more common in which population?

A

Ashkenazi Jews

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Niemann-Pick Disease (NPD) affects which organs significantly?

A

Spleen and liver

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Characteristic cell in Niemann-Pick Disease (NPD)?

A

Pick’s cell (Foam cell)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Description of Pick’s cell cytoplasm?

A

Swollen by many small lipid droplets

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Life expectancy in Niemann-Pick Disease (NPD)?

A

Often fatal by three years of age

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Gaucher Disease is caused by a defect or deficiency in what enzyme?

A

β-glucocerebrosidase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Most common lipidosis?

A

Gaucher Disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Inheritance pattern of Gaucher Disease?

A

Autosomal recessive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Characteristic cell in Gaucher Disease?

A

Gaucher Cell

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Features of Gaucher Cell cytoplasm?

A

Distended by glucocerebrosides

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Where is the Gaucher Cell typically found?

A

Bone marrow

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Description of Gaucher Cell nucleus?

A

Small, eccentric nucleus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Conditions where pseudo-Gaucher cells may be encountered?

A

Thalassemia, chronic myeloid leukemia, acute lymphoblastic leukemia, Non-Hodgkin lymphoma, plasma cell neoplasms

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Inheritance pattern of Chediak-Higashi syndrome?

A

Autosomal recessive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Key feature of Chediak-Higashi syndrome in phagocytes?

A

Large, abnormal cytoplasmic granules

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Granules in phagocytes (granulocytes and monocytes) in Chediak-Higashi syndrome are positive for what enzyme?

A

Peroxidase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Granules in lymphocytes in Chediak-Higashi syndrome are positive or negative for peroxidase?

A

Peroxidase (-)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What type of organelles are abnormally large in Chediak-Higashi syndrome?

A

Lysosomes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Reason for partial albinism in Chediak-Higashi syndrome?

A

Abnormal packaging of melanosomes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Physical characteristics associated with partial albinism in Chediak-Higashi syndrome?

A

Silvery hair, pale skin, photophobia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Triad of symptoms in Wiskott-Aldrich Syndrome (WAS)?

A

Thrombocytopenia, Immunodeficiency, Eczema

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Inheritance pattern of Wiskott-Aldrich Syndrome (WAS)?

A

X-linked recessive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What are the dysfunctional cells in Wiskott-Aldrich Syndrome (WAS)?
B cells, T cells, NK cells, neutrophils, and monocytes
26
Type of infections common in Wiskott-Aldrich Syndrome (WAS)?
Bacterial, viral, and fungal infections
27
Structural abnormalities in Wiskott-Aldrich thrombocytes?
Low number of dense granules and small size (microthrombocytes)
28
May-Hegglin Anomaly inheritance pattern?
Autosomal dominant
29
Key cytoplasmic feature of granulocytes and monocytes in May-Hegglin Anomaly?
Gray-blue spindle-shaped inclusions (Döhle body-like inclusions)
30
Hematologic findings in May-Hegglin Anomaly?
Leukopenia, variable thrombocytopenia, and giant platelets
31
Pelger-Huet Anomaly (PHA) is caused by a mutation in what gene?
Lamin β-receptor gene
32
Key function of lamin β-receptor protein?
Plays a major role in leukocyte nuclear shape changes during normal maturation
33
Characteristic nuclear shape in neutrophils with Pelger-Huet Anomaly?
Bilobed (spectacle-like 'pince-nez') or unilobed (round, ovoid, peanut-shaped)
34
Most common genetic disorder of WBCs?
Pelger-Huet Anomaly (PHA)
35
Inheritance pattern of Pelger-Huet Anomaly?
Autosomal dominant
36
Homozygous Pelger-Huet Anomaly neutrophil nuclei?
All neutrophils are round
37
Heterozygous Pelger-Huet Anomaly neutrophil nuclei?
55% to 93% of neutrophils affected, showing a mixture of nuclear shapes
38
Granulation pattern in neutrophils with true Pelger-Huet Anomaly?
Normal granulation
39
Functional status of neutrophils in true Pelger-Huet Anomaly?
Function normally
40
Another name for Acquired Pelger-Huet Anomaly?
Pseudo-Pelger-Huet Anomaly
41
Characteristics of neutrophils in pseudo-Pelger-Huet Anomaly?
Hyposegmented and hypogranular
42
Conditions associated with pseudo-Pelger-Huet cells?
Acute myeloid leukemia, chronic myeloproliferative neoplasms, myelodysplastic syndromes (MDS), HIV infection, tuberculosis, Mycoplasma pneumoniae, and severe bacterial infections
43
Defining feature of hypersegmented neutrophils?
Nucleus with > 6 lobes
44
Conditions associated with hypersegmented neutrophils?
Megaloblastic anemias, myelodysplastic syndromes, hereditary neutrophil hypersegmentation, myelokathexis
45
Key feature of neutrophils in myelokathexis?
Hypersegmentation, hypercondensed chromatin, pyknotic changes
46
Definition of myelokathexis?
Rare hereditary condition with normal granulocyte production but impaired release into the blood, leading to neutropenia
47
Lupus Erythematosus (LE) Cell is typically what type of cell?
A neutrophil that has ingested the antibody-coated nucleus of another neutrophil or a homogenous, globular nuclear mass
48
Primary condition associated with LE Cells?
Systemic lupus erythematosus (SLE)
49
Other disorders where LE Cells may be found?
Comparable connective tissue disorders
50
Tart Cell is typically what type of cell?
A monocyte that has ingested a whole lymphocyte or nucleus with identifiable nuclear chromatin
51
Condition where Tart Cells may be observed?
Drug sensitivity
52
Defining feature of Rieder Cell nucleus?
Notched, lobulated, cloverleaf-like
53
Conditions where Rieder Cells may be found?
Chronic lymphocytic leukemia or artificially formed through blood film preparation
54
Another name for Grape Cell?
Morula cell or Mott cell
55
Grape Cell cytoplasm is filled with what structures?
Russell Bodies (antibodies and immunoglobulins)
56
Disease associated with Grape Cells?
Multiple Myeloma (Plasma Cell Myeloma)
57
Key diagnostic protein in Plasma Cell Myeloma?
Bence Jones Protein
58
CRAB acronym in Plasma Cell Myeloma stands for?
Hypercalcemia, Renal insufficiency, Anemia, Bone lesions
59
Defining feature of Hairy Cells?
Small lymphocytes with little cytoplasmic projections
60
Hairy Cells test positive for which enzyme?
Tartrate resistant acid phosphatase (TRAP)
61
Disease associated with Hairy Cells?
Hairy Cell Leukemia
62
Which isoenzyme of acid phosphatase is abundant in Hairy Cells?
Isoenzyme 5
63
Definitive histologic characteristic of Hodgkin’s Disease?
Presence of Reed-Sternberg cells
64
Key features of Reed-Sternberg cells?
Large lymphoid cell with two nuclei, eosinophilic nucleoli, and abundant cytoplasm
65
Flower cells are seen in what condition?
Adult T-cell leukemia
66
Another name for Popcorn cells?
L and H cells
67
Condition associated with Popcorn cells?
Nodular Lymphocyte Predominant Hodgkin’s Lymphoma (NLPHL)
68
Characteristic feature of Sezary cells?
Cerebriform nucleus
69
Disease associated with Sezary cells?
Mycosis Fungoides (a Non-Hodgkin’s Lymphoma)
70
Toxic granulations are found in which type of cell?
Neutrophils
71
Appearance of toxic granulations?
Dark-blue to black granules in the cytoplasm
72
Conditions associated with toxic granulations?
Severe infections and chemical poisoning (e.g., lead poisoning)
73
Appearance of Auer rods in cells?
Linear projections of primary granules
74
Condition associated with Auer rods?
Certain types of acute Myelogenous Leukemia (AML)
75
Defining feature of a Faggot cell?
Abnormal WBC with bundles of Auer rods in its cytoplasm
76
Description of Dohle bodies?
Round or oval blue-staining cytoplasmic inclusions in neutrophils, arranged in parallel rows and consisting of ribosomal RNA
77
Conditions associated with Dohle bodies?
Pregnancy, severe burns, aplastic anemia, scarlet fever, infectious diseases, and administration of toxic agents
78
Additional feature associated with MHA inclusions?
Presence of giant platelets
79
Dohle bodies vs. MHA inclusions: Size?
Dohle: small; MHA: larger
80
Dohle bodies vs. MHA inclusions: Shape?
Dohle: round; MHA: spindle
81
Dohle bodies vs. MHA inclusions: PAS reaction?
Dohle: positive; MHA: negative
82
Dohle bodies vs. MHA inclusions: Content?
Dohle: rRNA; MHA: mRNA
83
MHA inclusions unique feature?
Giant platelets

HEMATOLOGY 1 (29 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions