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13 Cards in this Set

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What is the Difference


Morphologic Alt V Functional Abn


What are the Nuclear Abn?


What are the Cytoplasmic Abn?

Morphologic are problems with appearance such as problems within the nucleus or within the cytoplasm.


Functional These generally do not have physical changes to the appearance of the cell but function irregularly.


Nuclear - Pelger Huet , Hered Neut Hyperseg


Cyto - Alder-Reilly


Chediak Higashi


May-Hegglin


Fx Abn - Chronic Granulomatous Disease


Leuk Adh Disorder, Mono/Macro Lysosomal Storage Disease, B/T Lymph Abn,


Misc - Job Synd, Lazy Leuk Synd, MPO Def.



(Morph)Pelger-Huet Anomaly


Morph/Funct?


What Cell Line affected?


Dom/Rec?


How does it occur?


*Pseudo-Pelger-Huet Anomaly

Morphological Abn


Decrease Nuclear Segmentation (neuts) BILOBED


Autosomal Dom Disorder


*MOST COMMON GEN DISORDER


Mut. of Lamin B Receptor


*It is an occurance when >50% of Segs are affected Seen in Mal. Myeloproliferative Neoplasm.



(Morph)Hereditary Neut Hypersegmentation


What does the name mean?


Dom/Rec?


*Break it down*

Autosomal Dom Cond.
Dom

Autosomal Dom Cond.


Dom



(Morph)Alder-Reilly Anomaly


Dom/Rec


Appearance of cell?

Rec


Granulocytes are large dark purple resembles Toxic Granulation it is in all leukocytes

(Morph)Chediak-Higashi Syndrome


Dom/Rec


Appearance of Cell?


Patient Concerns?

Rec
Enlarged Lysosomal Vess, Giant Gray-Green Granules
Fatal Disease- Rare, Bleeding Disorders Bact Inf. Albinism and Immunodef associated.

Rec


Enlarged Lysosomal Vess, Giant Gray-Green Granules


Fatal Disease- Rare, Bleeding Disorders Bact Inf. Albinism and Immunodef associated.

(Morph)May-Hegglin Anomaly


Dom/Rec


Appearance of Cell?


Patient Concerns?

Dom


Large Baso Inclusions, Giant Plt, Thrombocytopenia, Leuks Resemble (Dohle Body)


Patients are Asymptomatic but can have bleeding disorders, thrombocytopenia

Chronic Granulomatous Disease CGD


Fx Abn?


Dom/Rec


Patient Concerns?

X Linked Recessive


Inability to phagocytosis to produce react O2 Spec, WBCs unable to function


Pre-Disp to Infections and Formation of Granulomas affects GI and Urinary

Differences Between


a. Leukocyte Adhesion Disorder


b. Job Syndrome


c. Lazy Leukocyte Syndrom


d. Myeloperoxidase Deficiency


e. Diabetes Mellitus

a. Inability of neuts and monos to bond to endothelial cells (Cell to Tissue Transport)


b. Elevated IgE, Skeletal Abn, Bact Inf.


c. Rare, Neutropenia poor responses to chemotactic agents.


d. Most Common Neutrophil Abn, Mild Sympt


e. Type I Inh, Type II Acq Associate with poor Neut func

Mono/Macro Lysosomal Storage Disease


What are the two divisions?


Mechanism?

Mucopolysaccharide Storage Disease


Lipid Storage Diseases


Inherited enzyme deficiencies or defects resulting in flawed degradation of phagocytize material.

What is lipid storage disease and what are some of the specific diseases associated with it and how do they work?

Defective Lipid Catabolism


Gaucher Disease - most common lipid storage disease Gaucher cells anemia


Neimann-Pick Disease - Def in acid sphingomelinase, neurologic problems as infant, marrow contains many macrophages

What are B/T Lymphocyte Abn & what is the difference between the each?

Genetic disorders that result in decreased prod of B/T cells

DiGeorge Syndrome Decreased number of T-Lymphs absence or underdevelopment of thymus


Comb B/T


Severe Combined Immunodeficiency - Decreased # of T/B cells and lack of NK cells- No Immune system.


Wiskott-Aldrich Syndrome- X linked mutation patients have immunodeficiency, eczema, and thrombocytopenia




What are the 5 quantitative abnormalities

Neutrophils - Neutrophilia, leukamoid reaction, leukoerythroblastic reaction, neutropenia


Eosinophils - eosinophilia


Basophils - basophilia


Monocytes - monocytosis


Lymphocytes -lymphocytosis, lymphocytopenia



What are the 3 qualitative disorders

Granulocytes Lift Shift - Mild, Mod, Marked


Monocytes - Imm Monos observer in infection response


Lymphocytes - Variant, reactive (confused with blast), atypical