WBC ANOMALIES Flashcards
Deficiency of SPHINGOMYELINASE (for breakdown of LIPIDS) ; rare autosomal recessive disease ; more commonly seen in ASHKENAZI JEWS ;
Niemann-Pick disease
A macrophage whose cytoplasm is swollen by many LIPID droplets
Pick’s cells (Aka foam cell)
Defect or deficiency in the catabolic enzyme Beta glucocerebrosidase ; cramped tissue paper or onion skin appearance ; most common of lipid ones ; an autosomal recessive disorder
Gaucher’s disease
Found in the bone marrow ; large macrophage with small eccentric nucleus ; cytoplasm is distended by glucocerebrosides
Gaucher cell
Can be encountered thalassemia, chronic myeloid leukemia, ALL, non-Hodgkin lymphoma, plasma cell neoplasm
Pseudo-Gaucher cells
A rare autosomal recessive disease of immune dysregulation ; characterized by the presence of LARGE abnormal cytoplasmic granules in phagocytes
Chediak-Higashi syndrome
Abnormal granules in phagocytes reaction to peroxidase
Positive
Abnormal granules in lymphocytes peroxidase reaction
Negative
Numerous types of cells in the body are affected and show abnormally LARGE LYSOSOMES, which contain fused dysfunctional granules ; PARTIAL ALBINISM
Chediak-Higashi syndrome
Basic defect is golgi complex that is responsible for granule assembly ; abnormal granules
Chediak-Higashi syndrome
A rare X-linked recessive disorder where T cells are decreased ; B cells, T cells, and NK cells are dysfunctional which leads to bacterial, viral, fungal infections ; thrombocytes are structurally abnormal ; the number of dense granules is low and the thrombocytes is low and SMALL (MICROTHROMBOCYTES)
WISKOTT-ALDRICH SYNDROME
Patient with Wiskott-Aldrich syndrome exhibit a triad of
Thrombocytopenia
Immunodeficiency
Eczema
An autosomal dominant disorder characterized by the presence of GRAY BLUE spindle shaped inclusions ; DOHLE BODY-LIKE inclusions in the cytoplasm of granulocytes and monocytes ; variable thrombocytopenia and GIANT PLATELETS
MAY-HEGGLIN ANOMALY
Failure of the neutrophil nucleus to segment ; most common genetic disorder of WBCs ; an autosomal dominant disorder with decreased nuclear SEGMENTATION, coarse chromatin clumping pattern potentially affecting all leukocytes
Pelger-Huet anomaly
“Pince-nez” or “spectacle” form of neutrophil nucleus ; hyposegmented
Pelger-Huet cell
Spectacle-like “pince-nez” morphology with nuclei attached by a thin filament
Bilobed nuetrophil
A result of a mutation in the laminate B-receptor gene
Pelger-Huet anomaly
An inner nuclear membrane protein ; plays a major role in leukocyte nuclear shape changes that occur during normal maturation
Latin B-receptor
All neutrophil are affected by and demonstrate round nuclei
Homozygous Pelger-Huey anomaly
55% to 93% of the neutrophil population are affected
Heterozygous PHA
Pelger-Huet anomaly is also known as
True congenital PAH
ACQUIRED PHA is also known as
Pseudo PHA
May be seen in acute myeloid leukemia, chronic myeloproliferative neoplasms and myelodysplastic syndrome, HIV infection, tuberculosis, mycoplasma pneumoniae, and severe bacterial infections ; resembles the neutrophil of Pelger-Huet cells
Pseudo-Pelger-Huet cell
Neutrophils show _______ in true PHA
Normal granulation
Number of lobes in hypersergmented neutrophils
6 or more lobes
Pertains to a rare hereditary condition characterized by normal granulocyte production; nevertheless, there is impaired release in to the blood (leads to neutropenia)
Myelokathexis
Usually a neutrophil that has ingested the antibody-coated nucleus of another neutrophil or has engulfed the homogenous, globular nuclear mass of a destroyed cell ; usually an in vitro phenomenon
Lupus erythematosus cell
A monocytes that has ingested a whole lymphocyte or a nucleus (With an identifiable nuclear chromatin) ; may be seen in DRUG SENSITIVITY
Tart cell
Similar to normal lymphocytes but the nucleus is notched, lobulated, and CLOVERLEAF-like ; found in CLL or can be artificially formed through blood film preparation
Reader cell
An abnormal plasma cell with a cytoplasm that is completely filled with Russel bodies ; found in multiple myeloma
Grape cell
Grape cell is also known as
Morula cell or Mott cell
Patients with plasma cell myeloma have these characteristics:
Presence of Bence Jones Proteins
CRAB (hypercalcemia, renal insufficiency, anemia, bone lesions)
CRAB stands for
Hypercalcemia
Renal insufficiency
Anemia
Bone lesions
Small lymphocytes with little cytoplasmic projections ; nearly all blood cells contain 7 nonerythroid isoenzymes of acid phosphatase (0,1,2,3b,4, and 5) ; TRAP positive
Hairy cells
TRAP stands for
Tartrate
Resistant
Acid
Phosphatase
Isoenzyme 5 is ____ and is produced in abundance by Hairy cells
Tartrate resistant
A LARGE LYMPHOID cell which may demonstrate two nuclei (with eosinophilic nucleoli) and an abundant cytoplasm ; owl’s eye appearance ; definitive histologic characteristic of HODGKIN LYMPHOMA
Reed Sternberg cell
Seen in adult T cell leukemia
Flower cells
Lymphocytic and histologic cells ; seen in nodular lymphocyte predominant Hodgkin’s lymphoma
Popcorn cells
Popcorn cells also known as
L and H cells
Characterized by a cerebriform nucleus ; seen in mycosis fungicides (an example of a Non-Hodgkin’s lymphoma
Sezary cells
Purple red particles (precipitated MUCOPOLYSACCHA RIDES) ; seen primarily in neutrophils, eosinophil, and basophil and occasionally in monocytes and lymphocytes ; can resemble very coarse toxic granulation
Alder-Reilly inclusions
Most frequently seen in patients with Hunter, Hurler, and Maroteaux-Lacy types of genetic MUCOPOLYSACCHARIDOSIS
Alder-Reilly inclusions
Altered primary granules (present because of rapid cell maturation) ; dark blue to black granules found in cytoplasm of neutrophils ; seen in lead poisoning
Toxic granulation
Linear projections of primary granules ; seen in AML
AUER RODS
Bundles of sticks ; an abnormal WBC with bundles of Auer rods in its cytoplasm
Faggot cells
Round or oval blue staining cytoplasmic inclusions found in neutrophils (arranged in parallel rows and consisting of ribosomal RNA)
Dohle bodies
Found in pregnancy ; severe burns, aplastic anemia, scarlet fever, toxic agents
Dohle bodies
Smaller, round, PAS positive with ribosomal RNA
Dohle bodies
Larger, spindle shape, PAS negative with messenger RNA content
Dohle body-like inclusions in May-Hegglin anomaly
