WBC-ANOMALY

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White Blood Cells Anomalies

Nuclear Abnormalities

  • Hypersegmented Neutrophil

    • Neutrophils with more than 5 lobes and larger than usual

    • Associated with deficiencies of Vitamin B12 and Folic acid

    • Related to Pernicious Anemia

  • Pelger-Huet Anomaly

    • Hyposegmented Neutrophil

    • Nucleus appearance: dumbbell, pair of glasses

    • Function: normal

    • Acquired autosomal dominant disorder

    • Benign Anomaly

  • Barr body

    • "Sex-Chromatin Bodies"

    • Small, round projection of nuclear chromatin

    • Represents the 2nd X-chromosome of females

    • Note: <10% of neutrophils among females

  • Giant Neutrophil

    • Larger than usual neutrophils

    • Can be occasionally seen in Normal Peripheral Circulation

    • Often observed in Hypersegmented Neutrophils

Cytoplasmic Abnormalities

  • Alder-Reilly Inclusions

    • Granules: Purple-red, precipitated mucopolysaccharides

    • Found in 1° Granulocytes, 2° Monocytes/Lymphocytes

    • Note: Larger than toxic granulation

    • Associated with Hurler's Syndrome and Hunter's Syndrome

  • Auer Rods

    • Reddish-purple, rod-like bodies

    • Represents primary granules

    • Normal: low number

    • Abnormal: high number

    • Associated with Acute Myelogenous Leukemia (AML)

  • Chediak-Higashi Syndrome

    • Impaired chemotaxis

    • Granules: Peroxidase (+), Large, Reddish-purple

    • Hereditary disease (autosomal recessive trait)

  • Dohle Bodies "Amato bodies"

    • Single/multiple 1° Neutrophil, 2° Monocytes/Lymphocytes

    • Seen near Periphery of cytoplasm

    • Wright stain: Light Blue

    • Represents aggregation of RER

    • Associated with Burns, Viral Infection, Certain Drug Therapy

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  • Toxic Granulation

    • Dark blue-black granulation cytoplasmic granules

    • Granules represent precipitation of RNA

    • Primary: Peroxidase (+)

    • Seen in Band and Segmented Neutrophils

    • Associated with Burns, Drugs therapy/Poisoning

  • Jordan's Anomaly

    • Vacuolation of cytoplasm of phagocytes (granulocytes, monocytes)

    • Results in aging and active phagocytosis

    • May reflect increased lysosomal activity

    • Associated with Fatty Degeneration and Glycogen

    • Associated with Septicemia, Severe infection

  • May-Hegglin Anomaly

    • Platelets and granulocyte disorder/anomaly

    • Observe: Giant Platelets, Thrombocytopenia

    • With Dohle-like Inclusions in granulocytes (larger)

Abnormalities of Cells Exhibiting Phagocytosis

  • LE cells

    • Neutrophil with large purple homogenous round inclusion with nucleus wrapped around

    • Appear smooth and evenly stained

    • Associated with Autoimmune disorders such as Lupus erythematosus

  • Tart cells

    • Monocyte with ingested lymphocyte

    • Appears rough & unevenly stained

Abnormalities Associated with Lymphocytes

  • Atypical Lymphocytes/Downey cells

    • Reactive lymphocytes, Variant lymphocytes, Transformed lymphocytes, Leukocytoid lymphocytes

    • Type I: Turks's Irritation cells/Plasmacytoid lymphocytes with large block chromatin

    • Type II: IM cells with round mass chromatin "Ballerina Skirt" appearance

    • Type III: "Swiss cheese" appearance, Moth-eaten appearance

    • Associated with Infectious Mononucleosis

  • Basket cell/Smudge cell

    • Squashed, degenerative nuclei of cells

    • Associated with Chronic Lymphocytic Leukemia (CLL)

  • Hairy cells

    • B lymphocytes with hair-like cytoplasmic projections surrounding the nucleus

    • TRAP (+)

    • Associated with Hairy cell leukemia

  • Sezary cells

    • Lymphocyte with T cell characteristics with grooved nucleus and brain-like convolutions ("cerebri" form)

    • Associated with Sezary syndrome, Mycosis fungoides, Cutaneous T-cell lymphoma

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Abnormalities Associated with Plasma Cells

  • Flame Cell

    • Red to pink cytoplasm associated with an increase in IgA

    • Associated with Multiple Myeloma

  • Russell Bodies

    • Individual globules of immunoglobulins

    • Associated with Multiple Myeloma, Reactive state

  • Grape Cell/Berry Cell/Morula Cell/Mott Cell

    • Vacuoles and large protein globules

    • Appear like grapes

    • Associated with Multiple Myeloma, Reactive state

  • Dutcher's Bodies

    • Intranuclear protein inclusions

Function Abnormalities

  • Job syndrome

    • Neutrophils exhibit normal random activity (chemokinesis) but abnormal directional activity (chemotaxis)

  • Lazy Leukocyte Syndrome

    • Neutrophils have abnormal random and directional activity

  • Chronic granulomatous disease

    • Inability of phagocytes to kill ingested microorganisms due to impaired NADPH oxidase/Respiratory burst

    • Nitroblue tetrazolium dye test

  • Leukocyte Adhesion Disorder-I (LAD-I)

    • Caused by decreased or truncated β2 integrin, needed for neutrophil adhesion to endothelial cells and recognition of bacteria

    • Leads to Recurrent Infection, Neutrophilia, Lymphadenopathy, Splenomegaly, Skin Lesions

  • Leukocyte Adhesion Disorder-II (LAD-II)

    • Caused by decreased amount or function of selectin ligands and defective leukocyte recruitment

    • Physical growth retardation, Coarse face, Neurological defect, Recurring infection

  • Absence of blood group H antigen

  • Leukocyte Adhesion Disorder-III (LAD-III)

    • Defective protein Kindlin-3, needed for β integrin activation and leukocyte rolling

    • Failed response to external signals that would normally result in leukocyte activation

    • Recurrent bacterial and fungal infection (less severe than LAD-I)

    • Decreased platelet GPIIbβ3 leading to bleeding

Monocyte/Macrophage Lysosomal Storage Disorders

  • Gaucher Disease

    • β-glucocerebrosidase deficiency

    • Accumulation of Glucocerebroside

    • Wrinkled/Crumpled Cytoplasm, "Cat-Scratch" Cytoplasm

    • Clinical Triad used in Diagnosis: Hepatomegaly, Gaucher cells in BM, Increased serum phosphatase

  • Niemann-Pick Disease

    • Sphingomyelinase deficiency

    • Accumulation of sphingomyelin

    • Foamy cytoplasm

  • Fabry Disease

    • α-galactosidase deficiency

    • Accumulation of Ceramide trihexose

  • Tay-Sachs Disease

    • Hexosaminidase A deficiency

    • Accumulation of GM2 ganglioside

    • Vacuolated cytoplasm

  • Sandhoff Disease

    • Hexosaminidase A and B deficiency

    • Accumulation of Glycolipid and ganglioside

    • Vacuolated cytoplasm

Mucopolysaccharidosis

  • MPS I-Severe Hurler Syndrome

    • α-l-iduronidase deficiency

    • Accumulation of Dermatan sulfate, heparan sulfate

  • MPS I-Attenuated Scheie Syndrome

    • α-l-iduronidase deficiency

    • Accumulation of Dermatan sulfate, heparan sulfate

  • MPS II-Severe Hunter Syndrome

    • Iduronate sulfatase deficiency

    • Accumulation of Dermatan sulfate, heparan sulfate

  • MPS III Sanfilippo Syndrome Type A

    • Heparan-N-sulfate accumulation

    • Heparan sulfate

  • MPS III Sanfilippo Syndrome Type B

    • α-N-acetylglucosaminidase deficiency

    • Heparan sulfate

  • MPS III Sanfilippo Syndrome Type C

    • Acetyl-coenzyme A:a-glucosaminide N-acetyltransferase deficiency

    • Heparan sulfate

  • MPS IV Morquio Syndrome Type A

    • Galactose-6-sulfatase deficiency

    • Accumulation of Keratan sulfate, chondroitin-6-sulfate

  • MPS IV Morquio Syndrome Type B

    • β-Galactosidase deficiency