Part 9: WBC anomalies

Niemann-Pick Disease

Deficiency of sphingomyelinase

Foam cell

characteristic cell of Niemann-Pick Disease

Niemann-Pick Disease

A macrophage where cytoplasm is swollen by many small lipid droplets

Gaucher Disease

Has a defect or deficiency in the catabolic enzyme: B-glucocerebrosidase

Gaucher Disease

Has a cytoplasm of “crumpled tissue appearance,” paper or onion skin like

Chediak-Higashi

Basic defect: Golgi complex

Gaucher disase

Partial albinism is observed in this anomaly

Thrombocytopenia

Immunodeficieny

Eczema

WAS exhibits the characteristic triad of

Wiskott-Aldrich Syndrome

A rare X-linked recessive disorder

microthrombocytes

What are the thrombocytes present in WAS?

May-Hegglin

An autosomal dominant with Dohle body-like inclusions

Pelger-Huet Anomaly

Failure of the neutrophil nucleus to segment

pince-nez or spectacle form

Identifying characteristic of Pelger-Huet Anomaly

Myelokathexis

Rare hereditary condition characterized by normal granulocyte production but impaired impaired release to the blood

LE cell

Neutrophil that has ingested the antibody-coated nucleus of another neutrophil

Tart Cell

A monocyte that has ingested a whole lymphocyte or a nucleus

Rieder cell

Nucleus is notched, lobulated, and cloverleaf-like

Grape cell

aka Morula cell or Mott cell

Grape cell

An abnormal plasma cell with a cytoplasm that is completely filled with Rusell Bodies

Hairy Cells

TRAP +

Isoenzyme 5

What isoenzyme is produced in abundance by Hairy Cells?

Reed-Sternberg Cell

Definitive histologic characteristic of Hodgkin’s Disease

Sezary cells

Cerebriform nucleus

Alder-Reilly

Appears in 4 types of WBCs

Toxic granulations

Seen in severe infections and chemical poisoning (lead)

Faggot cell

An abnormal WBC with bundles of Auer Rods in its cytoplasm