Non-Malignant Granulocytic Disorder Flashcards by Jimleo Badilla

Quantitative Differences in CBC results
AGE RELATED CHANGES

Pediatric Population:
-Ratio of Neutrophils to Lymphocytes changes with age

-Newborns have a HIGHER WBC count, Neutrophil % and absolute count

-By ___ year of age, the lymphocytes % is almost DOUBLE the neutrophil % - this is called a ‘reverse ratio’ or ‘inverted diff’

one

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Quantitative Differences in CBC results
AGE RELATED CHANGES

Geriatric Population
-Adult Ref Intervals based on younger population

-CBC results of healthy older people only SLIGHTLY different than younger adults

> 65y have slightly _____ WBC, RBC, HGB & PLTS & slightly ______ MCV

-As body ages some cells lose ability to divide (e.g muscle, nerve)

-Bone Marrow & Hematopoiesis slightly affected:
—–Less hematopoietic cells produced:
*Normal adult BM cellularity = __%
* >65y adult BM cellularity = __%
—–Probably due to loss of bone and increased BM fat stores

LOWER
HIGHER

50%
30%

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Quantitative Differences in CBC results
AGE RELATED CHANGES

Geriatric Population

Immune Response:
-Reduced signaling in some cells
*_______ and _______

-Loss of thymus – must rely on memory & tissue T-cells
*T-cells regulate B-cells = less antibody production
*More susceptible to infections

lymphocytes and neutrophils

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Quantitative Differences in CBC results
AGE RELATED CHANGES

Geriatric Population

-More prone to various anemias:
*IDA & Megaloblastic
*Anemia of chronic disease (Rheumatoid arthritis)

-More prone to cancers
-Leukemias
-Most hematological disorders have increased incidence with age

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Quantitative Neutrophil Changes

Absolute Neutrophilia

__________
-Shift in Neuts from Marginal into Circulatory pool
—–Stress, Trauma, Labor, strenuous exercise, shock, burns or increase in epinephrine

*Pathological COnditions
-Shift in Neuts from Marginal into Circulatory pool
-Increased BM production
-Release of Neutrophils from Storage Pool to PB
—infections

Benign

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Quantitative Neutrophil Changes

Absolute Neutrophilia

TERMINOLOGIES:

______ _______- describes an increased number of immature cells as an indicator of infection
-Increase in bands, metamyelocytes (possibly myelocytes)
-Increased release from storage pool
-Usually seen with neutrophilia and toxic changes

Left Shift

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Quantitative Neutrophil Changes

Absolute Neutrophilia

TERMINOLOGIES:

________ _____________: A reactive leukocytosis above 50 × 10^9/L with NEUTROPHILIA and a MARKED LEFT SHIFT
-Bands & often metas/myelos
-Could have rare Pro or Blast

-are mostly a result of:
*Severe &/or chronic infection (e.g. TB, pneumonia)
*Metabolic disease
*Inflammation
*Response to a malignancy

Leukemoid reaction

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Quantitative Neutrophil Changes

Absolute Neutrophilia

TERMINOLOGIES:

______________ _________: Presence of IMMATURE NEUTROPHILS, NUCLEATED RED BLOOD CELLS, and TEARDROP RBCs in the same sample
-Often accompanied by neutrophilia, but not always
-point to the possibility of a space-occupying lesion in the bone marrow:
*Metastatic tumor
*Fibrosis
*Lymphoma
*Leukemia
*or simply a marked increase in one of the normal marrow cells (e.g., erythroid hyperplasia seen in hemolytic anemia)
*is strongly associated with primary myelofibrosis.

-Presence of immature erythrocytes & increased neutrophils
-Not always a neutrophilia – could be normal count with early cells

Leukoerythroblastic picture

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Quantitative Neutrophil Changes

Absolute Neutropenia

Causes:
1. Increased rate of removal or destruction
2. Decreased production or ineffective hematopoiesis
3. Decreased ratio of circulating vs. marginal pool
4. Depletion of BM storage pool
5. BM suppression – decreased production\impaired release:
*Some Acute Leukemias
*Aplastic anemia

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EOSINOPHILS AND BASOPHILS

Eosinophilia- Absolute count > ____ x 10^9/L

causes:
-Allergies (asthma)
-Parasitic infection
-Some auto immune disorders (HIV)
-Fungal infections
-Some malignancies (ALL)

0.4

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EOSINOPHILS AND BASOPHILS

Basophilia - Absolute count > ___ x 10^9/L

-Non Malignant causes:
*Bee stings
*Food/drug hypersensitivity
*Chronic infections
*Hypothyroidism
*Chronic Inflammation
*Radiation therapy

-Malignant myeloproliferative neoplasms:
*Chronic Myelogenous Leukemia

0.15

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Qualitative Granulocyte Disorders

Qualitative Abnormalities

Acquired Granulocyte Alterations & Anomalies:

NUCLEUS
-Hypersegmentation
-Hyposegmentation
-Pyknotic and Necrobiotic forms

CYTOPLASM
-Toxic granulation
-Degranulation
-Vacuolization
—–with or without engulfed matter
-Dohle bodies

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Acquired Qualitative Nuclear Disorders

-nucleus have over 5 segments
-chronic infections
-megaloblastic anemia
-drugs

Hypersegmented

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Acquired Qualitative Nuclear Disorders

-bilobed or no segmentation
-Myelodysplastic syndromes
-Asynchrony of nuclear maturation:
——very clumped chromatin
——no segmentation

Hyposegmented

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Acquired Qualitative Nuclear Disorders

-‘dying’ cell
-nucleus verydark/dense
-Filaments still visible

Pyknotic

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Acquired Qualitative Nuclear Disorders

-dead cell
-no filaments

Necrotic

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_________ cells
-Usually only seen in BM
-Not abnormality – just a phase
-Comment if several seen
-Don’t count in diff – cannot identify stage

Mitotic cells

Acquired Qualitative Cytoplasmic Disorders

Neutrophilic Cytoplasmic Abnormalities

-Absence of granules
——Hypo- or Agranular
——‘Degranulation’
-Abnormal granules

Toxic Changes in Neutrophils

While these changes may be considered “abnormal,” they usually reflect a normal, reactive response

__________ _______________:
-Stress response to infection / inflammation
-Clinically significant
—–Marker of inflammation

-Report with WBC morphology
—–OCC/SOME/MANY

Toxic Granulation

Toxic Changes in Neutrophils

2.___________ ___________:
-Neutrophils contain several vacuoles
—–often seen with toxic granulation
——some spontaneously occur
-can indicate phagocytosis
——may contain bacteria and other material

*Report with WBC morphology
—-OCC/SOME/MANY

Toxic Vauolization

Toxic Changes in Neutrophils

3.________ _________:
-Pale blue, oval inclusions at periphery
—–Grey – light blue on Wright stain
—–Only in Neutrophils
-Remnants of ribosomal RNA
-Associated with
—-Bacterial infection
—-sepsis
—–pregnancy

*Report with WBC morphology
—-OCC/SOME/MANY

Dohle Bodies

Ingested Materials – Neutrophils

identify:

Platelet satellitism

Ingested Materials – Neutrophils

identify:

Histoplasma capsulatum

Ingested Materials – Neutrophils

identify:

Bacilli

Ingested Materials – Neutrophils identify:

Yeast

LE Cell Phenomenon Lupus Erythematous (LE) Cell -a mature neutrophil which has phagocytized a spherical, homogeneous-appearing inclusion -Inclusion is nuclear material of degenerating leukocytes coated with antinuclear antibody -associated with SLE & some other autoimmune conditions *Not seen in PB unless sample is manipulated -can be seen in body fluids (serous and synovial fluid) -Neutrophil segments are stretched around the phagocytose nucleus -The protein in the engulfed nucleus is depolymerized by the antibody and appears homogenous -LE Cell test previously used to diagnose SLE

Cytoplasmic Abnormalities ______ _______ -Not a non-malignant change -Only seen in Blasts and Promyelocytes in acute leukemia ------Only seen in Myeloblasts – not lymphoblasts -Fused Primarygranules ------Azurophilic rods not to be confused with Dohle bodies

Auer rod

INHERITED QUALITATIVE ABNORMALITIES ______-_____ _________ -Hyposegmentation of neutrophil nucleus -Autosomal Dominant -Asymptomatic (fully functional) *________ state: -Bi-lobed nucleus -Dense heterochromatin -Incomplete nuclear segmentation -aka“dumbbell” or “pince-nez *_______ state: -Nucleus is round or oval -No segmentation *Nuclear chromatin is exceptionally coarse and condensed – it is a mature cell *Could be confused with Band or Myelocyte *Pelger-Huet - 70 to 90% neutrophils affected

Pelger-Huet Anomaly Heterozygous Homozygous

INHERITED QUALITATIVE ABNORMALITIES "______-_______-______": -induced by drugs -seen in patients with HIV, TB and Mycoplasma pneumonia, severe bacterial infections -secondary to myelodysplastic syndromes, acute leukemias & chronic myeloproliferative disorders

Pseudo-Pelger-Huet

INHERITED QUALITATIVE ABNORMALITIES True VS Pseudo-Pelger-Huët _____: all WBCs are affected (nuclear shape and chromatin structure) ______: usually only affects Neutrophils and is accompanied by other abnormal changes (i.e. Neutrophilia, toxic changes) Reported in comments: Pelger-Huët forms (if all of the neutrophils are affected – classic bi-lobed or single-lobed forms Quantified under WBC Morph: Hyposegmented neutrophils (if they appear dysplastic, i.e. mature chromatin but no segmentation)

True Pseudo

INHERITED QUALITATIVE ABNORMALITIES _____-______ _________ -Autosomal Recessive -Abnormally large metachromatic granules in the cytoplasm of: *Granulocytes *Monocytes *Lymphocytes -Incomplete degradation of mucopolysaccharides ----------Mucopolysaccharide deposits (granules) in most cells -Leukocyte function is unaffected -Deep-purple to lilac granules appear in WBCs -In some cases only one cell line is involved -May resemble promyelocytes or heavy toxic granulation -----Neutrophilia, Dohle bodies and left shift = toxic granulation -----Alder-Reilly = granules can be found in lymphocytes and monocytes *very rare

Alder-Reilly Anomaly

INHERITED QUALITATIVE ABNORMALITIES ________-________ ________ -Inclusions similar to Döhle bodies except: *Seen in all granulocytes & monocytes *Composed of precipitated myosin heavy chains *Larger than ones in infection and permanent, not transient *Not seen with other toxic changes *Giant and bizarre platelets, may be hypogranular *Patients are usually asymptomatic or have mild bleeding disorders

May-Hegglin Anomaly

INHERITED QUALITATIVE ABNORMALITIES ________-_________ __________ -Autosomal Recessive -Rare, fatal -Characterized by abnormal fusion of granules in most cells that contain granules throughout the body -Fused granules are large and mostly dysfunctional -Hematopoietic cells are affected -Disease manifestations can be found in hair, skin, adrenal and pituitary glands, and nerves -Dysfunctional phagocytosis & inefficient bacterial destruction -------Increased susceptibility to infection (immunodeficient) albinism and peripheral neuropathy -Platelet dense granules ------Tendency to bleed -Melanocytes: ----Variable albinism -Neurologic complications develop through childhood

Chediak-Higashi Syndrome

TO RECAP MORPHOLOGICAL ABNORMALITIES OF NEUTS Morph Changes: decreased nuclear segmentation in neuts; sometimes also affects other WBCs Clin findings: asymptomatic

Pelger-Huet Anomaly

TO RECAP MORPHOLOGICAL ABNORMALITIES OF NEUTS Morph Changes: decreased nuclear segmentation in neuts Clin findings: depends on underlying condition

Pseudo-Pelger-Huet

TO RECAP MORPHOLOGICAL ABNORMALITIES OF NEUTS Morph Changes: >5 nuclear lobes in neutrophils Clin findings: depends on underlying cause

Neutrophil hypersegmentation

TO RECAP MORPHOLOGICAL ABNORMALITIES OF NEUTS Morph Changes: granulocytes contain large, darkly staining metachromatic cytoplasmic granules Clin findings: normal neutrophil function. clinical findings, if present are due to associated condition

Alder-Reilly anomaly

TO RECAP MORPHOLOGICAL ABNORMALITIES OF NEUTS Morph Changes: Giant lysosomal granules in granulocytes monocytes, and lymphocytes Clin Findings: leukocyte dysfunction and recurrent pyogenic infections; bleeding due to abnormal dense granules in platelets

Chediak-Higashi disease

TO RECAP MORPHOLOGICAL ABNORMALITIES OF NEUTS Morph Changes: Thrombocytopenia, giant platelets and large Dohle body-like inclusions in neutrophils, eosinophils, basophils, and monocytes Clin findings: usually asymptomatic; sometimes mild bleeding related to the degree of thrombocytopenia

May-Hegglin anomaly

_______ ___________ _________ -Rare X-linked or Autosomal Recessive ------Neutrophil Morphology is usually normal ------Decreased ability of phagocytes to produce NADPH oxidase (kills microorganisms) -Recurring bacterial & fungal infections ------Bacteria are not killed on ingestion ------Remain encased in granulomas - localize bacteria ------These may rupture and release live bacteria and pus Morphology: -Normal WBC morph or Neutrophilia with toxic changes (as seen in bacterial infection) -Use ______ _________ reduction test or ____ __________ to diagnose this condition

Chronic Granulomatous Disease NItroblue tetrazolium