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31 Cards in this Set

  • Front
  • Back

Autosomal dominant ,dominantly inherited defect of terminal neutrophil differentiation due to defect in nucleic acid metabolism

Pelger huet anomaly

Morphology of pelger huet anomaly

Nucleus fail to undergo segmentation,it fails to form more than 2 segments

Heterozygous form of pelger huet anomaly

Hyposegmented,bilobed,spectacle -pence nez nuclei

Homozygous form of pelger huet anomaly

Rare ,neutrophil contain single round eccentric nuclei with clumped chromati n and little segmentation

Function of pelger huet anomaly

Normal neutrophilic function/natural resistance to infection

Benign autosomal dominant

Hereditary hypersegmentation

Morphology

Five or more nuclear lobes -single 6 lobes of 100 cell dif

Autosomal recessive trait multisystem disease

CHA

Due to defective function ,chemotaxis

Recurrent bacterial infection

Due to abnormal granules in melanocyte

Oculocutaneous albinism

Due to platelet granule defect

Mild bleeding disorders

Morphology CHA

Large lysosomal cytoplasmic granules

Rare autosomal dominant abnormality -non muscle myosin heavy chain 9 (chromosome 22)

May hegglin anomaly

Clinical for may hegglin

Most patient are clinically asymptomatic

Dohle body like inclusion

May hegglin anomaly

Thrombocytopenia with giant abnormal platelets

May hegglin anomaly

Association of hunter and hurler syndrome

Alder Reilly anomaly

Lack lysosomal enzyme to break mucopolysaccharide

Alder Reilly anomaly

Dense azurophilic granules in leukocyte

Alder Reilly

Metachromatic granules seen in leukocyte surrounded by clear zone seen in lymphocyte

Alder reilly

Can be differentiated from inherited by presence of >10% of normal 3 lobed neutrophil

Pseudo pelger huet anomaly

Seen in severe inflammatory states

Toxic granulation

is thought to be due to impaired cytoplasm maturation in effort to rapidly generate large number

Toxic granulation

Toxic granulation are azurophilic that are found in

Promyelocyte,metamyelocyte,band,segment

Single multiple blue cytoplasmic inclusion

Dohle bodies

Associated with myeloid left shift and seen in conjunction with toxic granulocyte

Dohle bodies

Represent remnant's of rough endoplasmic reticulum

Dohle bodies

Seen in burns surgery and infection

Dohle bodies

Larger than may hegglin anomaly

Dohle bodies

Implies that phagocytosis has occured

Degranulation

May mean degeneration ,implies than phagocytosos

Vacuolization