Lecture 1.3: WBC Disorders

Question 1

Which 7 drugs, hormones, or toxins which are common causes of acute neutrophilia?

Answer 1

• Corticosteroids
• Epinephrine
• Lithium
• Endotoxin
• Smoking
• Vaccines
• Venoms

Question 2

What are some common causes of demargination syndrome leading to neutrophilia?

Answer 2

• Vigorous exercise
• Acute physical and emotional stress
• Infusion of epinephrine and other catecholamines

Question 3

Shift of neutrophils from the marginal to circulating pool is quantitatively more important from which vascular bed and can account for about a doubling in neutrophil count?

Answer 3

Pulmonary capillaries

Question 4

An increase in which cells in the blood can be helpful in distinguishing neutrophilia due to demargination from other causes such as infections, protracted stress, or glucocorticoid administration?

Answer 4

↑ in lymphocytes + monocytes + neutrophils

Question 5

Acute neutrophilia occurs as a consequence of release of neutrophils from where; this type of neutrophilia is seen in response to what?

Answer 5

Marrow storage pool; in response to inflammation and infections

Question 6

Inflammation or tissue necrosis, due to burns, trauma, gout, or vasculitis are causes of what type of neutrophilia?

Answer 6

Acute neutrophilia

Question 7

Malignancy is a cause of what type of neutrophilia?

Answer 7

Chronic Neutrophilia

Question 8

Asplenia and myeloproliferative disorders are causes of what type of neutrophilia

Answer 8

Chronic neutrophilia

Question 9

If neutrophilia persists after repeat labs, what is the next step (flow chart)?

Answer 9

• Examine smear: Leukoerythroblastic?
• Yes: then proceed to BM examination: morphology, cytogenetics, culture
• No: determine if pt is febrile (if yes, evaluate and treat), if not then do serologies for AID

Question 10

If blood smear of pt with neutrophilia shows leukoerythroblastic cells, what are you looking for with the BM examination and what are the DDx’s?

Answer 10

• Tumor granulomatous dx
• Ph¹ or BCR-ABL —> CML
• JAK2 mutation –> Non-CML MPN = PCV or myelofibrosis

Question 11

Smoking and obesity can be associated with what WBC abnormality?

Answer 11

Mild neutrophilia

Question 12

Lymphocytes displaying coarse, clumped chromatin, in pt with lymphocytosis should raise suspicion of which primary marrow disorder?

Answer 12

CLL

Question 13

Readily identifiable lymphoblasts in pt with lymphocytosis should raise suspicion of which primary marrow disorder?

Answer 13

ALL

Question 14

An excess of large granular lymphocytes, particularly in a pt with RA, should raise suspicion of which disorder?

Answer 14

Large granular lymphocytic (LGL) leukemia

Question 15

Why is examination of the blood lymphocytes in pt with lymphocytosis so important?

Answer 15

Lymphocyte morphology is generally more informative than is the case in neutrophilia

Question 16

Larged lymphocytes with cleaved nuclei may be seen in which cause of lymphocytosis?

Answer 16

Follicular lymphoma

Question 17

Pt presenting with rhinosinusitis, asthma, and eosinophilia should trigger screening for what?

Answer 17

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Question 18

List 3 medications which are common causes of eosinophilia?

Answer 18

• Antibiotics
• NSAIDs
• Antipsychotics

Question 19

List 5 allergic diseases associated with eosinophilia

Answer 19

• Allergic rhinitis
• Atopic dermatitis
• Urticaria/angioedema
• Fungal allergy
• Asthma

Question 20

Unexplained monocytosis, particularly in elderly patients with other cytopenias, should reflex concern for what; which work-up is generally warranted?

Answer 20

• Myeloid malignanacies, such as MDS and CMML
• Generally warrants examination of bone marrow

Question 21

List 6 antibiotics associated with idiosyncratic neutropenia?

Answer 21

• Cephalosporins
• Clindamycin
• Gentamicin
• Sulfonamides
• Tetracyclines
• Vancomycin

Question 22

List 3 anticonvulsants associated with idiosyncratic neutropenia

Answer 22

• Carbamazepine
• Mephenytoin
• Phenytoin

Question 23

Which antidepressant is associated with idiosyncratic neutropenia?

Answer 23

Amitriptylene

Question 24

List two H2 blockers associated with idiosyncratic neutropenia.

Answer 24

• Cimetidine
• Ranitidine

Question 25

List 3 cardiovascular drugs associated with idiosyncratic neutropenia

Answer 25

- Hydralazine - Captopril - Propranolol

Question 26

List 2 diuretics associated with idiosyncratic neutropenia

Answer 26

- Chlorothiazide - Hydrochlorothiazide

Question 27

Which drug commonly used to prevent gout is associated with idiosyncratic neutropenia?

Answer 27

Allopurinol

Question 28

Which antipsychotic medication is associated with idiosyncratic neutropenia?

Answer 28

Chlorpromazine

Question 29

Which 2 infections are commonly associated with chronic neutropenia?

Answer 29

**HIV** and **Hepatitis**

Question 30

What is Felty's Syndrome? \*likely on exam\*

Answer 30

**Hyper**splenism w/ **neutropenia**, due to **long-standing RA**

Question 31

What is the management for a pt with neutropenia, fever, localizing signs, and ANC \<500/mL?

Answer 31

**Admit** for **IV antibiotics** and consider **G-CSF**

Question 32

A pt with a history of neutropenia whom also has a postive family hx or with no family hx but cyclic neutropenia should be worked up for what?

Answer 32

**ELANE mutation** --\> the gene coding for neutrophil elastase, seen in **cyclic neutropenia**

Question 33

What is the one of the most common causes of iatrogenic lymphopenia?

Answer 33

Glucocorticoids

Question 34

List 4 congenital immunodeficiency diseases associated with lymphopenia?

Answer 34

- Severe combined immunodeficiency disease (**SCID**) - Common variable immune deficiency - Ataxia-telangiectasia - Wiskott-Aldrich syndrome

Question 35

List some of the acquired causes of lymphopenia?

Answer 35

- **Aplastic anemia** - **Infectious diseases:** viral, bacterial, and parasitic - **Iatrogenic:** immunosuppressive agents and chemotherapy

Question 36

What is the most notable entity to exclude in a patient monocytopenia and constitutional sx's + splenomegaly?

Answer 36

Hairy cell leukemia

Question 37

Which 2 markers for Hairy Cell Leukemia can be seen via flow cytometry?

Answer 37

**CD11c** and **CD103**

Question 38

Severe monocytopenia may be seen in which syndrome; due to what mutation?

Answer 38

- Seen in **MonoMAC** (monocytopenia and mycobacterial infection) **syndrome** - Mutation in the **GATA-2 gene** - **High risk** of progressing to **MDS** or **AML**