NAME: __________________________________________________ WRITTEN_______ MORPH ________

QUIZ | Chapter 62 - Classification and Clinical Manifestation of Neutrophil Disorders

Chapter 63 Neutropenia and Neutrophilia

1. Chronic cyclic neutropenia is characterized by periodic oscillations in the number of neutrophils, with the nadir occurring at approximately at
______-week intervals
A. 1 C. 3
B. 2 D. 4

2. Neutrophilia exceeding _______ × 109 neutrophils/L has been designated a “leukemoid reaction,” if not a myeloproliferative neoplasm, and
reflects an underlying inflammatory, infectious, or neoplastic cause.
A. 25 C. 100
B. 50 D. 200

3. Leukemoid reaction not related to a clonal myeloid disease is described as follows EXCEPT:
A. Composed largely of mature neutrophils with a high proportion of bands and myelocytes
B. Has increased leukocyte alkaline phosphatase reaction in neutrophils
C. Has normal cytogenetics and genetics of marrow cells
D. Has cytometric analysis of neutrophils indicating a cluster of differentiation (CD) 13 and CD15 phenotype with absent expression of
human leukocyte antigen-D related (HLA-DR) and CD34

4. For individuals older than age 10 years, neutropenia is defined as a count less than approximately _____ × 10 9/L
A. 1.0 C. 1.6
B. 1.5 D. 1.8

5. The majority of patients with sporadic or autosomal dominant severe congenital neutropenia have heterozygous mutations of the gene for:
A. ELANE C. GATA2
B. HAX1 D. CXCR4

6. Aside from Barth Syndrome, this is another X-linked congenital neutropenia:

A. Severe Congenital Neutropenia with ELANE gene mutation
B. Severe Congenital Neutropenia with WAS gene mutation
C. Chediak Higashi Syndrome
D. Cohen Syndrome

7. G-CSF is a very effective therapy for severe congenital neutropenia as it acts to increase the neutrophil counts by enhancing expression of a
critical transcription factor for granulopoiesis which is:
A. RUNX1 C. C/EBPβ
B. GCSFR D. Neutrophil elastase

8. TRUE OR FALSE: Pure White Cell Aplasia (0.25 each)

A. It is a rare acquired disorder causing severe selective neutropenia.
B. The marrow is devoid or nearly devoid of neutrophils and their precursors
C. Thymoma, large granular lymphocyte syndrome, autoimmune diseases, ibuprofen, chlorpropamide, excessive zinc, and various
infectious and inflammatory diseases are considered possible causes
D. Immunosuppressive therapy with cyclosporine, sirolimus, antithymocyte globulin, and glucocorticoids have been used successfully
in individual cases

9. TRUE OR FALSE: Chronic Idiopathic Neutropenia (0.25 each)

A. Predominantly affecting young adult men ages 18–35 years; the female-to-male ratio is approximately 1 to 8
B. Erythrocyte, reticulocyte, and platelet counts usually are low.
C. Quantitative marrow studies show the ratio of immature to mature cells is decreased suggesting, ineffective granulocytopoiesis.
D. All three types of antibodies are present, including antinuclear or antimitochondrial antibodies, are present

10. Autoimmune neutropenia is associated in this condition described as the presence of splenomegaly, deforming rheumatoid arthritis, and
leukopenia:
A. SLE C. Felty Syndrome
B. Sjogren Syndrome D. Hemansky Pudlak Sydrome

11. What mineral deficiency can cause neutropenia in patients on total parenteral nutrition, patients with a history of gastrectomy, and
malnourished children, and the bicytopenia or tricytopenias with a marrow showing dysplastic precursors?
A. Selenium
B. Copper
C. Zinc
D. Cobalt

12. TRUE regarding Drug-induced Neutropenia EXCEPT

a. It is an idiosyncratic drug reaction.
b. Symptomatic patients with drug-induced neutropenia usually present with fever, myalgia, and sore throat but usually no rash or
evidence of allergy elsewhere.
c. Often an increase in the blood lymphocyte count heralds marrow recovery, and an “overshoot” with marked neutrophilia follows.
d. Treatment usually consists of supportive care, including broad-spectrum antibiotics for febrile patients.

13-15. MATCHING TYPE: Mechanism of neutrophilia (0.5 each)

i. Exercise A. Decrease Marrow storage pool shift
ii. Chronic Disorders (infections, tumors, endocrinopathies)
B. Decrease Marginal pool shift
iii. Corticosteroids (2 answers)
iv. Endotoxins C. Decrease Egress from circulating pool
v. Postneutropenia D. Increase proliferation
E. Increase proliferation + Increase Peripheral Granulocyte
Survival
 vi. Chronic Myeloid Leukemia

16. TRUE OR FALSE | PSEDONEUTROPHILIA (0.25 EACH)

A. It can be caused by vigorous exercise and acute physical and emotional stress.
B. This response in humans is dependent partially on release of neutrophils from the lungs and liver
C. With these conditions, neutrophil counts are elevated, while lymphocyte and monocyte counts are increased as well. 
D. The response is caused by a shift of cells from the marginal to the circulating pool; hence, it frequently is referred to as
demargination.

17. An unusual dermatologic condition manifested as intense neutrophil accumulation in the skin and persistent neutrophilia
A. Leukemia Cutis C. Cutaneous Leukocytoclastic Vasculitis 
B. Pyoderma gangrenosum D. Sweet Syndrome

18. Neutrophilia is associated in the following drugs except:

A. Epinephrine D. Ranitidine
B. Lithium Salts E. None of the above
C. Minocycline

19. Measurement of leukocyte alkaline phosphatase activity can be a useful screening test in cases of moderate neutrophilia. Values are
______________ with inflammation and in subjects receiving glucocorticoid therapy and ____________ in chronic myelogenous leukemia.
A. Increased, Decreased C. Increased, Increased
B. Decreased, Increased D. Decreased, Decreased

20. TRUE OR FALSE In myeloproliferative neoplasms, neutrophilia is a predictor of thrombotic events.

BONUS: MAX OF 1 POINT

1. What does MASCC mean (scoring for febrile neutropenia)?
2. What MASSC score is considered LOW RISK? (Clue: Duncan, Garnet)
3. Give 2 brands of GCSF

MORPH EXAM: