Usera: Benign WBC Abnormalities Flashcards by Megan Foggia

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

bone marrow production and release
survival time in the blood or rate of transport to tissue
ratio of marginated to circulating neutrophils

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What constitutes neutrophilia in adults?

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

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

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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Causes of neutrophilia?

acute inflammation
acute infection
tissue necrosis
drugs, toxins, metabolic
physiologic
neoplastic

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What are two features associated with REACTIVE neutrophilia?

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

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

leukemoid reaction

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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

leukoerythroblastic reaction

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What cell count constitutes neutropenia?

less than 2/L in whites

absolute neutrophils <1.3 in blacks

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What cell count constitutes agranulocytosis?

<0.5/L

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3 mechanisms for neutropenia?

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

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Causes of neutropenia?

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

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

True

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

Fanconi’s anemia

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What kind of hypoplasia occurs in Fanconi’s anemia?

panmyeloid –> all cells in the bone marrow are underproduced

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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

Kostmann’s syndrome

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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

cyclic neutropenia

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What are the two most common “congenital” neutropenias?

pregnancy induced hypertension

infection

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

old specimen
WBC fragility
paraprotein

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What does a neutrophil look like with toxic granulation?

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

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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

Dohle bodies

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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

vacuolization

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

Pelger-Huet Anomaly

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

hypersegmentation

What is hypersegmentation of neutrophils associated with?

megaloblastic anemia (B12 deficiency)
chronic infection
myelodysplastic syndromes

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

Alder-Reilly anomaly

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

Chediak-Higashi

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

May-Hegglin anomaly

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

chronic granulomatous disease

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

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

What test is used to check for chronic granulomatous disease?

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

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

myeloperoxidase deficiency

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

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

Popular finding in leukocyte adhesion deficiency?

delayed separation of the umbilical cord

What constitutes eosinophilia in a patient?

absolute eosinophils > 0.45/L

Causes of eosinophilia? Most common cause?

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

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

eosinophilic leukemia

Pulmonary infiltrate with eosinophilia syndrome | Tropical eosinophilia

Löffler's syndrome

What do corticosteroids do to eosinophil level?

DECREASE them bc they sequester in lymph nodes

What cell count constitutes basophilia?

>0.2/L

Causes of basophilia?

``` immediate hypersensitivity reactions chronic myeloproliferative disorders CML basophilic leukemia irradiation ```

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

0. 2 - 0.8 | 0. 8 is monocytosis

Causes of monocytosis? (too many monocytes)

``` chronic inflammation hematologic disease ** monocyte disorders infectious recovery state post-splenectomy ```

Causes of monocytopenia? (low monocytes)

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

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

Gaucher disease

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

>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)

``` 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 ```

acute infectious lymphocytosis

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

persistent polyclonal B cell lymphocytosis

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

infectious mononucleosis (Mono)

Absolute lymphocyte count <2.0 x 109/L in children

lymphocytopenia

Causes of lymphocytopenia? (low lymphocytes)

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

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

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

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

Wiskott Aldrich Syndrome

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

DiGeorge Syndrome

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

X-linked agammaglobulinemia

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

Hereditary Ataxia-Telangiectasia