General Information Flashcards
This is a Nonmalignant Leukocyte disorder that is autosomal dominant characterized by hyposegmentation of the granulocytes (most apparent in neutrophils).
Pelger Huet Abnormality
Pseudo Pelger-Huet cells are found in patients
during or after chemotherapy
Does Pelger Huet abnormality cause disease?
No
This is a Nonmalignant Leukocyte disorder that is autosomal dominant and clinically insignificant. It is characterized by hypersegmentation of the neutrophil.
Hereditary Hypersegmentation Anomally
How can you differentiate between Hereditary Hypersegmentation and Megaloblastic Anemia?
Megaloblastic Anemia will have oval macrocyts, hypersegmentation, and an MCV ? 120.
This Nonmalignant Leukocyte Disorder is very rare and characteized by cells that are a mirror image with axel symmetry. It is aquired during malignancies and chemotherapy.
Twinning Anomaly
This Nonmalignant Leukocyte disorder can be caller “drumstick” or “chicken leg”, and is believed to be a reminant of an inactivated X chromosome in women.
Nuclear Extrusion anomaly
Increased numbers of these cells are linked to genetic problems
Nuclear Extrusion anaomaly cells
This disorder is characterized by large or dominant primary granules. 1 of 3 toxic changes. Neutrophils can look like eosinophils, and they are clinically significant. Watch for staining errors.
Toxic granulation
This disorder is a clinically significant reminant of phagocytosis. Is is 2/3 of the toxic changes and is formed by incomplete digestion and degridation of bacteria and fungi.
Vaculolization
This leukocyte disorder occurs when neutrophils accumulate RNA. 3/3 of the toxic changes. Can be in bands, segs, monos, ect.
Dohle Bodies
Dohle Bodies are caused by a stimulating event, such as
Burns Infections Surgery Pregnancy Use of Granulocyte Monocyte Colony Stimulating Factor (GMCS)
These 3 toxic changes are always clinically significant and always reported.
Toxic Granulation
Vacuolization
Dohle Bodies
This disorder has cells that have small pyknotic chuncks, characteristic of a neutrophil that dies and falls apart. Low numbers are NOT clinically significant, but large numbers are.
Necrobiosis
This is a recessive anomally characterized by decreased mucoplysaccharide. Can be found in granulocytes. Can barely see nucleus because of prominent, heavy granulation.
Alder-Reily Anomally
This anomally can be seen in Hunters or Hurlers syndrome
Alder-Reily Anomally
This is a rare autosomal recessive disease characterized by giant, lysosomal granules in cells. It is genetic and fatal (bone marrow transplant sometimes). Patient has recurrent bacterial infections.
Chediak-Higashi Syndrom
This rare autosomal dominant disorder has large Dohle-like bodies which are RNA fibrils. Often accompanied by thrombocytopenia but many patients are asymptomatic. Patients are at risk for infections and mucutaneous hemorrhage. Bleeding episodes reported.
May-Hegglin Anomally
This disease is familial and heterozygous. Cells develop granulomas when they cannot complete process of phagocytosis. Reoccurent bacterial/fungal disease.
Chronic Granulomatous Disease of Childhood
How is Granulomatous Disease of Childhood diagnosed?
Nitoblue tetrazolium (NBT): measures respiratory burst needed for phagocytosis
Gangliosidosis, Tay-Sachs, Fabry, Aquired hyperlipidemia, Gauchers, and Neimann Picks disease are all due to
abscence of a particular enzyme which leads to accumulatied products
This is the most common storage disease and is autosomal recessive. It affects macrophages. Enzyme deficiency leads to build up of glucocerebroside (lipid).
Gaucher Disease
Type 1 Gauchers is found in and characterized by
Ashkenazi- No CNS involvement, symptoms asymptomatic to sever
Type 2 Gauchers is found in and characterized by
Acute neuropathic- CNS involvement, much more sever then type 1
