Sect 6 - WBC Disorders

< 1 week

lifespan of neutrophils

2 weeks

lifespan of eosinophils

12-18 months

lifespan of basophils

years

lifespan of T-cells

few months

lifespan of B-cells

3 days

lifespan of monocytes

Myeloblast

Promyelocyte

Myelocyte

Metamyelocyte

Band

Segmented Neutrophil

6 stages of neutrophil maturation

Myeloblast

cytoplasm: blue

no granules

nucleoli present in nucleus

Promyelocyte

Nucleus: round or oval

Nucleoli: present in nucleus

Cytoplasm: pale blue with dark-purple to red-purple

Non-specific primary granules

Myelocyte

Nucleus: circular-shaped

No nucleoli present

Granules: specific secondary granules present

last stage of myeloid cell division

Metamyelocyte

Nucleus: kidney-bean shaped

Granules: pink-purple present in cytoplasm

Band

Nucleus: horse-shoe shaped

Granules: pink-purple present in cytoplasm

Segmented neutrophil

Nucleus: segmented

Granules: pink-purple present in cytoplasm

storage pool

marginal pool

circulating pool

WBCs can be found in 3 places:

storage pool

WBCs found in bone marrow

marginal pool

WBCs found in walls of the blood vessels in lungs, liver and spleen

circulating pool

WBCs found in peripheral blood

conditions with Neutrophilia

Leukemoid reactions

Bacterial infections

Chronic myelogenous leukemia (CML)

Myelofibrosis

Pregnancy

conditions with Neutropenia

Viral illnesses

Malaria

Thyroid disorders

Aplastic anemia

Typhoid fever

conditions with Eosinophilia

Parasitic infections

Allergic reactions

Connective tissue disorders

Asthma

CML

Hodgkin's lymphoma

Polycythemia vera

Myelofibrosis

conditions with Eosinopenia

Shock

Trauma

Surgery

Administration of Corticosteroids/Epinephrine

conditions with Basophilia

Allergic reaction

Basophilic leukemia

Polycythemia vera

CML

Myelofibrosis

conditions with Basopenia

Generally not an issue

Granulocytopenia

Granulocytosis

Leukemias

3 Granulocyte Disorders

Granulocytopenia

too few granulocytes/myelocytes

causes of Granulocytopenia

1. Reduced granulopoiesis with myeloid hypoplasia

2. Increased ineffective granulopoiesis

3. Reduced granulocyte survival

combo of all three mechanisms

What is the most common cause of Granulocytopenia?

Leukemoid reaction

Increased WBC count that can mimic leukemia, usually caused by bacterial infection

LAP score >118 units

Granulocytosis

too many granulocytes/myelocytes

Acute and Chronic

2 types of Granylocytosis

Acute Granulocytosis

increase granulocytes in a quick amount of time -- rxn happens in a matter of min

stress, exercise, administration of epinephrine

3 causes of Acute Granulocytosis

mechanisms of Acute Granulocytosis

increased marrow storage pool shift (coming out of bone marrow into circulation)

increased marginal pool shift

Chronic Granulocytosis

increase granulocytes caused by recurrent or sustained physiological stimulus

ex: pregnancy, pinworm

mechanisms of Chronic Granulocytosis

increased proliferation and production from the bone marrow

Increased granulocyte survival

Decreased movement from the circulating pool

Leukemias

abnormal cells

Eosinophilia

NAACP is related to:

Neoplasms

Allergic Reactions

Asthma

Connective Tissue Disorders

Parasitic infections

What does NAACP stand for?

heparin and histamine

The blue/black granules of basophils contain

Basophilia

Allergic reaction

Basophilic leukemia

Polycythemia Vera

Chronic Myelogenous Leukemia (CML)

Myelofibrosis

lab tests to evaluate Granulocytosis

Bacterial, Viral, Ricketsial Parasitic studies

CBC

Bone marrow

LAP

Chromosome studies

Pelger-Huet

May-Hegglin Anomaly

Chediak-Higashi Syndrome

Alder-Reilly Anomaly

Chronic Granulomatous Disease

Name the five inherited WBC disorders

neutrophils

What cell are the 5 WBC disorders commonly found in?

Pelger-Huet

cells called Pence-Nez

no hematological disorders

no physical characteristics

inherited autosomal dominant

hypo-segmentation

characteristics of neutrophil in Pelger-Huet

Pelgeroid

an acquired form of Pelger-Huet that can be induced by drugs or seen in leukemia (not true inherited)

May-Hegglin anomaly

anomaly that presents with low WBC, low and deficient PLTs

inherited autosomal dominant

Dohilod bodies & giant bizarre platelets

Inclusion that can be identified in May-Hegglin anomaly

Mild to severe hemorrhagic disease (bleeding and epistaxis)

No physical characteristics

Clinical features of May-Hegglin anomaly

Chediak-Higashi syndrome

the ineffective killing of bacteria due to impaired release of enzymes by enlarged granules

CHS1 gene

gene mutation associated with Chediak-Higashi syndrome

inherited autosomal recessive

Clinical features of Chediak-Higashi syndrome

Partial ocular cutaneous albinism

Seizures

Immunocompromised

Recurrent infections

WBC and platelet count decreased

Lab Features of Chediak-Higashi Syndrome

death usually occurs before early teens from recurrent pyogenic infections

What is the prognosis of Chediak-Higashi syndrome?

abnormal granulation of both granulocytic and lymphocytic cells

Chediak-Higashi syndrome morphology

primary and secondary granules fuse together

Granulocytic cell characteristics seen in Chediak-Higashi syndrome

azuerophilic granules in NK cells

Lymphocytic cell characteristics seen in Chediak-Higashi syndrome

Alder-Reilley anomaly

caused by a metabolic disorder of mucopolysaccharides

autosomal inheritance -- very rare

Hurlers

other name for Alder-Reilly

characteristics of Alder-Reilly anomaly

Lysosomal storage disorder with giant granules in neutrophils, lymphocytes, and monocytes

Associated with gargoylism

Chronic Granulomatous Disease

Condition where bacteria are ingested but not killed

Cells can't produce hydrogen peroxide in conjunction with other oxygen metabolites which are necessary to kill microorganisms

inherited autosomal recessive or sex-linked recessive

catalase positive organisms

CGD is associated with infections caused by

Catalase positive organisms

Staph

Salmonella

Nocardia

Pseudomonas

Listeria

Aspergillus

Candida

E. coli

Serratia.

micro-abscess and granuloma formation

CGD infections are characterized by

septicemia or chronic pulmonary disease in early childhood

What is the common cause of death in Chronic Granulomatous Disease?

Nitro Blue-Tetrazolium Test

What does NBT stand for?

NBT

Semiquantitative assay that tests phagocytic function of polymorphonuclear leukocytes

Normal: cells reduce colorless NBT into blue-black deposit within the cell

negative/no color change

results in NBT for a patient with CGD