WBC variants/abnormals Flashcards
Benign congenital hyposementation of PMNs
No granulocytes with >2 nuclear lobes
Pelger-Huet anomaly
Deficiency in enzymes to break down mucopolysaccharides accumulate in WBCs
Prominent red to purple granules may appear in all WBC types
May be difficult to distinguish from toxic granulation
Alder-Reilly inclusions/anomaly
Rare autosomal recessive disorder characterized by giant lysosomes in most cells of the body
Abnormally formed lysosomal granules in WBCs
Decreased platelets with abnormal large granules & defective function
Chediak-Higashi Syndrome
Dohle-like bodies that are not RNA but precipitated myosin heavy chains
Decreased platelets with giant or bizarre forms
May-Hegglin Anomaly
Neutrophil disorder where PMNs can phagocytize bacteria but not kill them
Defect in enzyme NADPH oxidase
Chronic Granulomatous Disease
Neutrophils have decreased enzyme myeloperoxidase, resulting in longer killing time of bacteria
Myeloperoxidase (MPO) Deficiency
High glucose levels result in abnormal oxidative burst
Diabetes mellitus
Inability of neutrophils & monocytes to adhere to endothelial cells and migrate from blood to tissues
Increased and potentially lethal bacterial infections
Leukocyte Adhesion Disorders
Cells don’t respond properly to chemotactic factors and have poor directional and random movement
Patients have history of recurrent infections
Lazy Leukocyte Syndrome
Malignancy with few to many “hairy cells” (hair-like cytoplasmic projections)
Hairy Cell Leukemia
Malignant cells that appear as mature lymphs with convoluted, cerebriform nuclear folds
Sezary Cells
Inability to degrade glucocerebroside, leading to accumulation in monocytes and macrophages
Deficient enzyme is beta-glucocerebrodase
Macrophage cytoplasm have crumpled silk/striated/chicken scratch appearance due to accumulation of lipids
Gaucher’s Disease
Inability to degrade sphingomyelin, which accumulates in liver, spleen, lungs, brain, bone marrow
Deficient enzyme is sphingomyelinase
Macrophages have “foamy cell” appearance
Niemann-Pick Disease
Accumulation of unmetabolized ganglioside in almost all tissue
Deficient in enzyme Hexosaminidase A
Cells have vacuolated lymph appearance
Tay-Sachs Disease
Deficient enzyme is beta-glucocerebrodase
Gaucher’s Disease
