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Block 7 Week 1 > Usera: Benign WBC Abnormalities > Flashcards

Usera: Benign WBC Abnormalities Flashcards

1
Q

What are 3 factors that affect the neutrophil concentration in the blood?

A
  1. bone marrow production and release
  2. survival time in the blood or rate of transport to tissue
  3. ratio of marginated to circulating neutrophils
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2
Q

What constitutes neutrophilia in adults?

A

neutrophils > 7 (E^9)/L in adults

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3
Q

What is the difference b/w immediate, acute, and chronic neutrophilia?

A

immediate: 20-30 minutes; redistribution from marginated to circulating pool
acute: 4-5 hours; release from marrow storage pool to blood
chronic: days; increase in marrow mitotic pool

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4
Q

Causes of neutrophilia?

A 
acute inflammation
acute infection
tissue necrosis
drugs, toxins, metabolic
physiologic
neoplastic
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5
Q

What are two features associated with REACTIVE neutrophilia?

A

shift to the left in myeloid maturation (Bands - not fully mature)
morphological alterations in neutrophils and precursors

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6
Q

A benign leukocyte proliferation with WBC usually >50 x 109/L with many circulating immature leukocyte precursors; Blasts are occasionally present

A

leukemoid reaction

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7
Q

Characterized by presence of nucleated RBC and a shift to the left in granulocyte maturation; Often associated with myelophthisic processes, severe hemorrhage, hemolytic anemia, or myelodysplastic syndromes

A

leukoerythroblastic reaction

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8
Q

What cell count constitutes neutropenia?

A

less than 2/L in whites

absolute neutrophils <1.3 in blacks

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9
Q

What cell count constitutes agranulocytosis?

A

<0.5/L

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10
Q

3 mechanisms for neutropenia?

A

decreased or ineffective marrow production
increased cell loss or neutrophils moving into tissues
pseudoneutropenia

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11
Q

Causes of neutropenia?

A 
drugs *like clozapine
intrinstic defects
overwhelming infection
hematologic disorders
autoimmune
debilitated states
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12
Q

T/F: Infections associated w neutropenia can be viral, bacterial, rickettsial, or protozoal

A

True

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13
Q

This is a heterogenous childhood disease caused by chromosomal instability; presents with aplastic anemia and congenital physical malformations

A

Fanconi’s anemia

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14
Q

What kind of hypoplasia occurs in Fanconi’s anemia?

A

panmyeloid –> all cells in the bone marrow are underproduced

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15
Q

Infantile genetic congenital agranulocytosis in which ANC <200/uL; variable modes of inheritance; early myeloid precursors in marrow, but they do not mature; associated with ELA2

A

Kostmann’s syndrome

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16
Q

Rare autosomal dominant trait that presents in infancy or childhood and causes period fluctuation in neutrophil count; it is associated with infection, fever and malaise during the neutropenic period; also associated with ELA2 gene mutation

A

cyclic neutropenia

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17
Q

What are the two most common “congenital” neutropenias?

A

pregnancy induced hypertension

infection

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18
Q

What are some reasons for false, or “spurious” neutropenia?

A

old specimen
WBC fragility
paraprotein

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19
Q

What does a neutrophil look like with toxic granulation?

A

large, blue-black granules; primary granules retain basophilia; Dohle bodies and vacuolization

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20
Q

Remnants of free ribosomes and rough endoplasmic reticulum; Seen in severe bacterial infections, pregnancy, burns, cancer, aplastic anemia, and toxic states; Often occur with toxic granulation and vacuolization

A

Dohle bodies

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21
Q

End stage of phagocytosed material, or fat or other substance; Often associated with Döhle bodies and toxic granulation; Often a predictor of sepsis; May be artifactual in stored blood

A

vacuolization

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22
Q

Autosomal dominant trait occurring in 1:5,000 people; Clinically ASYMPTOMATIC with normal neutrophil function; Heterozygous form
Homozygous form; Distinguish from reactive left-shift

A

Pelger-Huet Anomaly

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23
Q

Definition: >5% neutrophils with five lobes or any with six

A

hypersegmentation

24
Q

What is hypersegmentation of neutrophils associated with?

A

megaloblastic anemia (B12 deficiency)
chronic infection
myelodysplastic syndromes

25
Q

Autosomal recessive trait; Large, purplish GRANULES in cytoplasm; Granules stain metachromatically with Toluidine blue; Associated with mucopolysaccharidoses (Hunter’s syndrome, Hurler’s syndrome)

A

Alder-Reilly anomaly

26
Q

Autosomal recessive trait; Giant gray-green peroxidase- positive bodies in cytoplasm of leukocytes and other cells; Defects in fusion of cytoplasmic membranes, locomotion, and chemotaxis; Abnormal melanosomes (skin hypopigmentation, photophobia); Lymphadenopathy and hepatosplenomegaly

A

Chediak-Higashi

27
Q

Autosomal dominant trait; Large Döhle body-like inclusions in granulocytes; Involves myosin heavy chain 9; Associated with thrombocytopenia and GIANT OVAL PLATELETS with few granules; Typically present with BLEEDING disorder due to platelet defect

A

May-Hegglin anomaly

28
Q

Sex-linked and autosomal recessive inheritance patterns
Defect in RESPIRATORY BURST oxidase system
Presents in childhood with recurrent infections with low-grade pathogens
Formation of granulomas when neutrophils phagocytose, but do not kill, organisms

A

chronic granulomatous disease

29
Q

Patients with chronic granulomatous disease are more prone to infection by what organisms? Why?

A

catalase + bactertia, because catalase + bugs can break down H202 from the respiratory burst and can survive its toxic effects

30
Q

What test is used to check for chronic granulomatous disease?

A

Nitroblue Tetrazolium Test

**normal neutrophils produce H202 and )2- which will turn yellow NBT to BLUE; CDG neutrophils cannot reduce NBT

31
Q

Autosomal recessive inheritance
Absence of myeloperoxidase in neutrophils and monocytes (takes H202 –> HOCl)
Infections not a usual complication

A

myeloperoxidase deficiency

32
Q

Leukocyte adhesion deficiency involved low levels of what coreceptor? How does type 1 differ from type 2 and type 3?

A

CD18;
type 1: deficiency in CD18
type 2: deficiency in selectins on endothelial cells
type 3: failure to ACTIVATE CD11a/CD18

33
Q

Popular finding in leukocyte adhesion deficiency?

A

delayed separation of the umbilical cord

34
Q

What constitutes eosinophilia in a patient?

A

absolute eosinophils > 0.45/L

35
Q

Causes of eosinophilia? Most common cause?

A 
most commonly idiopathic
INVASIVE parasites
allergic disorders (asthma)
infections
addison's disease
malignancies
collagen disease
leukemia
36
Q

Persistent absolute eosinophil count >1.5 x 109/L in adults with tissue infiltration and no apparent cause

A

eosinophilic leukemia

37
Q

Pulmonary infiltrate with eosinophilia syndrome

Tropical eosinophilia

A

Löffler’s syndrome

38
Q

What do corticosteroids do to eosinophil level?

A

DECREASE them bc they sequester in lymph nodes

39
Q

What cell count constitutes basophilia?

A

> 0.2/L

40
Q

Causes of basophilia?

A 
immediate hypersensitivity reactions
chronic myeloproliferative disorders
CML
basophilic leukemia
irradiation
41
Q

What is the normal range for monocytes? Monocytosis vs monocytopenia?

A
  1. 2 - 0.8

0. 8 is monocytosis

42
Q

Causes of monocytosis? (too many monocytes)

A 
chronic inflammation
hematologic disease **
monocyte disorders
infectious
recovery state
post-splenectomy
43
Q

Causes of monocytopenia? (low monocytes)

A

stem cell disorders (aplastic anemia)
hairy cell leukemia
glucocorticoid therapy

44
Q

This in an inherited functional abnormality of monocytes, particularly macrophages; glucocerebrosidase deficiency leads to big sea foam blue macrophages in bone marrow, lymph nodes, liver, and spleen; serum acid phosphatase increased

A

Gaucher disease

45
Q

At what level are lymphocytes too high? In which cells do most changes in lymphocyte count occur?

A

> 4/L or >9/L in kids;
most changes in lymphocyte count are in T cells (T cells account for 60-80% of peripheral blood lymphocytes - mostly CD4 T cells)

46
Q 
CONTAGIOUS, MAINLY OCCURRING IN CHILDREN
PERSISTENT HIGH WBC COUNT
VARIABLE SYSTEMIC MANIFESTATIONS 
COXSACKIE VIRUSES A, B6, ECHOVIRUS, ADENOVIRUS
12-21 DAY INCUBATION
LYMPHOCYTOSIS LASTS 3-5 WEEKS
A

acute infectious lymphocytosis

47
Q

uncommon event; should raise suspicion for a chronic leukemia; persistent high levels of B cells; diagnose with flow cytometry

A

persistent polyclonal B cell lymphocytosis

48
Q

SELF-LIMITED, USUALLY UNNOTICED (3-5 WK INCUBATION)
VIRUS GAINS ENTRY VIA CD21 (RECEPTOR), INFECTS OROPHARYNGEAL MUCOSA AND LYMPHOID TISSUE
SORE THROAT, MALAISE, LYMPHADENOPATHY, SPLENOMEGALY
WBC 12-25 X 109/L
ATYPICAL LYMPHOCYTOSIS
POSITIVE HETEROPHIL ANTIBODY

A

infectious mononucleosis (Mono)

49
Q

Absolute lymphocyte count <2.0 x 109/L in children

A

lymphocytopenia

50
Q

Causes of lymphocytopenia? (low lymphocytes)

A 
destructive - radiation, chemo
debilitative - starving, cancer, anemia
infectious
AIDS
congenital immunodeficiency- wiskott aldrich
abnormal lymph circulation
51
Q

Who most commonly gets SCID? What are the symptoms of SCID?

A

males (75%); recurrent infections, failure to thrive, deficient T and B cells

52
Q

X-linked recessive inheritance (Xp11.3-Xp11.22)
Eczema, thrombocytopenia, and immunodeficiency
Increased risk of secondary neoplasm
Progressive decrease in thymus-dependent immunity
Absent antibodies to blood group antigens
Abnormal antibody production by B cells
No mitogenic response to CD43
May present with abnormal bleeding in neonatal period

A

Wiskott Aldrich Syndrome

53
Q 
Absence or hypoplasia of thymus
Hypoparathyroidism
Congenital heart defects
Dysmorphic facies
Hypocalcemia
Normal B cell function
Pathogenesis: del(22)(q11.2)
A

DiGeorge Syndrome

54
Q

Frequent respiratory and skin infections
Xq21.3-22
Block in B cell maturation at pre-B cell stage due to failure of variable and constant regions of IgM to connect
Decreased B lymphocytes and absent plasma cells
Marked decrease in serum immunoglobulins

A

X-linked agammaglobulinemia

55
Q

Autosomal recessive inheritance
Progressive ataxia, immune dysfunction, increased risk of malignancy
Defect in cell-mediated immunity with thymic hypoplasia or dysplasia
Diagnosis: increased chromosome breakage, t(14;14)
Gene product functions in DNA repair

A

Hereditary Ataxia-Telangiectasia

Block 7 Week 1 (9 decks)

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