leukocytes & non-malignant leukocyte disorders

what are granulocytes? Name 3

A group of leukocytes that have granules in their cytoplasm and have a segmented nucleus.

• eosinophils

• neutrophils (PMN’s)

• basophils

What cells come from the myeloid progenitor?

• granulocyte-monocyte progenitor

• megakaryocyte progenitor

• Erythrocyte progenitor

What cells come from the lymphoid progenitor?

All lymphocytes

• Dendritic cell

• B cells (B helper cells)

• T cells (T killer cells)

• NK killer cells

Put the following in order according to neutrophil maturation:

• segmented neutrophil

• myeloblast

• band

• promyelocyte

• myelocyte

• metamyelocyte

1. myeloblast

2. promyelocyte

3. myelocyte

4. metamyelocyte

5. band

6. segmented neutrophil

What is this cell according to neutrophil maturation and where would you see it?

myeloblast in bone marrow

What characteristics would you see in a myeloblast?

• slightly basophilic cytoplasm

• fine granular chromatin

• two to four visible nucleoli

What is this cell according to neutrophil maturation and where would you see it?

promyelocytes in the bone marrow

What characteristics would you see in a promyelocyte?

• nucleus is round to oval and is eccentric.

• a paranuclear halo or “hof” is seen

• cytoplasm is evenly basophilic and full of primary azurophilic granules.

pneumonic: what does the p for promyelocyte stand for?

It stands for primary azurophilic granules

what are the primary granules from a promyelocyte made up of?

• myeloperoxidase

• acid b-glycerophosphatase

• cathepsins

What is this cell according to neutrophil maturation and where would you see it?

myelocyte seen in bone marrow

What characteristics would you see in a myelocyte?

• cells have secondary granules

• secondary neutrophilic granules spread throughout the cell until it is more lavender-pink than it is blue

What is this cell according to neutrophil maturation and where would you see it?

metamyelocyte normally seen only in the bone marrow

What characteristics would you see in a metamyelocyte?

• nucleus starts to indent and looks like a kidney bean shape or peanut shaped

• chromatin is increasingly clumped

• tertiary granules may begin to appear

what is the difference between banded and segmented neutrophils?

presence of 2-5 nuclear nuclear lobes in segmented neutrophils

B lymphocytes develop in the ___.

Bone marrow

T lymphocytes develop in the _____.

Thymus

what type of cell is this that contains Azorophilic granules?

Activated NK cell

What is the classification, causation, and presentations of the Wiskott-aldrich syndrome?

Classification: combine immunodeficiency disorder

Causation: X-linked disease by mutation in the WAS gene —> decrease in WASp protein

Presentation: neutrophils and monocytes are dysfunctional which leads to bacterial and fungal infections

What is the causation and presentations of the Chediak Higashi syndrome?

causation: mutation in the CHS1 LYST gene on chromosome 1q42.1-2 that regulates the morphology and function of lysosome related organelles

presentation:

• cells exhibit giant lysosomal fused granules

• fused granules result in leukocyte dysfunction

• patients often have bleeding issues and recurrent pyogenic infections

What is the classification, causation, and characteristics of pelger huet anomaly (PHA)?

classification: true or congenital PHA is an autosomal dominant disorder

causation: mutation in the lamin B receptor

Characteristics:

• decreased nuclear segmentation (bilobed/unilobed)

• coarse chromatin clumping pattern

• seen in neutrophils and leukocytes

• needs to be 70-80% in peripheral blood to be pelger huet

What is the cause and morphology of Alder-Reilly anomaly?

Cause: transmitted as a recessive trait

Morphology: granulocytes with large, darkly staining metachromatic cytoplasmic granules composed primarily of partially digested monopolysaccharides.

What is the classification, cause, and morphology of may-hegglin anomaly?

classification: a rare autosomal dominant platelet disorder

cause: a mutation in the MYH9 gene on chromosome 22q12-13

morphology:

• variable thrombocytopenia

• giant platelets

• large dohle body-like inclusions in neutros, baso, eos, and mono

Seen in septic patients

What is the classification, cause, and morphology of Gaucher disease?

classification: most common lysosomal lipid storage disease

cause: defect/deficiency in the catabolic enzyme b-glucocerebrosidase (gene located at Iq21), which is necessary for glycolipid metabolism.

morphology: bone marrow contains gaucher cells, macrophages occuring individually or in clusters, that have an abundant fibrillar blue-gray cytoplasm with striated or wrinkled appearance.

What is the classification, characterization, and morphology of Niemann-pick disease?

classification: an autosomal recessive lipid storage disease

Characterization: type a and b are identified by the recessive mutations in the SMPD1 gene, which leads to a deficiency in the lysosomal hydrolase enzyme acid (sphingomyelinase ASM)

morphology: macrophages with a foamy cytoplasm packed with lipid-filled lysosomes that appear as vacuoles after staining bone marrow aspirate

What does leukomoid reaction mean?

reactive leukocytosis above 50×10^9 L with neutrophilia and a marked left shift (presence of immature neutrophilic forms). It is non-malignant.

what does leukoerythroblastic reaction mean?

the presence of immature neutrophils, nucleated RBCs, and teardrop RBCs

What is the formula for absolute neutrophil count (ANS)?

(WBC) x total neutrophils (segs% + bands%) x 10 = ANS

What is the one test that would help distinguish leukomoid reaction from chronic myeloid leukemia?

Leukocyte alkaline phosphatase (LAP score) would be elevated for leukomoid reaction but would be decreased for chronic myeloid leukemia

What is neutropenia and what does it cause?

It is the decrease in the absolute neutrophil count (ANC) and it raises the risk of severe infections. Two forms of getting neutropenia: acquired (drugs) and immune

What are some causes of non-malignant lymphocytosis?

• infectious mononucleosis

• hepatitis

• acute HIV infection

• chicken pox

• or just viruses

What are dohle bodies, in which cells are they found, and what do they look like?

Dohle bodies are cytoplasmic inclusions consisting of remnants of ribosomal ribonucleic acid (RNA).

typically found in neutrophils that often contain toxic granulation

they appear in the cytoplasm as pale blue round or elongated bodies.

What does toxic granulation of neutrophils look like?

• toxic granulation appears as dark, blue-black granules in the cytoplasm

• associated w/ infection

• a lot of granules but not pack the cytoplasm

when is vacuolation of neutrophils seen?

• seen in septic patients caused by either bacteria or fungi

what are some clinical manifestations and cause of infectious mononucleosis?

cause: by epstein-barr virus (EBV)

manifestations:

• sore throat, dysphagia, fever, chills, cervical lymphadenopathy, fatigue, and headache

• WBC elevated or with absolute lymphocytosis