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What is the def. of WBC anomalies?
congenital or hereditary condition where the WBC's deviate from the normal standard
what is the Etiology of Inherited (true) Pelger-huet?
- -autosomal dominant
- -abnormal maturation of nucleus
- -appear to function normally
What are the laboratory features of an Inherited (true) Pelger-huet?
- -decreased segmentation of nucleus
- -coarse and condense chromatin
this cell maybe drug induced, is secondary to leukemia(CmL) and is 10% normal 3 lobed nucleus
this cell is shift to the left, metamyelocytes and bands are present and is a response to severe infection
this is a defect in the lysomes and it leads to delayed degranulation(stained blue-purple granules)
this cell is autosomal dominant
What are the laboratory features of a may-hegglin
- -dohle bodies(blue straining area made up of rough plasma eticular & RNA)
- -giant, bizarre platelets
this cell is rare sex-linked recessive, fatal due to infetion, and defect in WBC; unable to kill catalase positive organism
What are the laboratory features of CGD
- -normal WBC morphology
- -WBC increases during infection
- -granulomas in many organs
- *granular tumor growth
- *sites of chronic infection
this disease is the deficiency of enzyme beta-glucocerebroside
what are the laboratory findings of Gaucher's disease
- -in bone marrow looks like unfolded crumpled paper
- -increased serum acid phosphatase
what is the etiology of tay-sachs disease?
- -autosomal recessive
- -deficiency oof hexaminosidase
- -seen in mostly jews
what is hexaminosidase?
accumulation of sphingolipids in lymphocytes
this disease death will occur within a few a years, and is a major site of pathology is central nervous system
what is the etiology of a neiman-pick?
- -aka Sea-blue histiocytes
- -due to defiency in sphigomyelinase
- (leads to accumulation of sphigomyelin in histocytes)
this the deficiency in enzymes that break down mucopolysaccharide
Hunter's & Hurler's
this cell is caused by EBV(Epstein Barr Virus)-infects the B-lymphocytes, affects all races mostly children and young adults, enter the body orally
What are the symptoms of infectious monocucleosis?
- -general malaise
- -enlarge lymph nodes
- -benign illness
what is the etiology of Leukemoid reaction?
- -neutrophilic response to infection
- -accelerated release of neutrophil
- -mimics early stage of leukemia
- (hence leukemoid reaction)
this has dark blue-black granules seen in severe infection (toxic granulation)
explain the Leukomoid reaction vs. CML
- WBC count---10-100---30-500
- Philadelphia chromosome---absent---present
this is the malignat disease of hematopoietic tissue
this is normal bone marrow elements replaced by neoplastic cells, bone marrow is always involved, and infiltates spleen, liver, lymph nodes
explain the acute vs. chronic
- age:------all ages----adults
- anemia--mild to severe--mild
this will become mild-severe when acute and mild when chronic
what are the laboratory features of acute leukemia?
- -WBC count:variable
- -WBC differential: present and usually predominate blast
- -normocytic, normochromic anemia if liver and spleen not affected
what is the purpose of FAB classification
to attain better discrimination in therapy and prognosis
what are the basis of FAB classification
- -WBC morphology
- -cytochemical staining
- -immunologic marker studies
explain the basis of FaB classification
- wbc morphology:
- -----------AML Blast vs ALL Blast
this is prominent AML blast usually >2
this indistinct ALL Blast usually 2 or less
this is present in 50-60% in AML Blast
this is never present in ALL Blast
this is the presence or absence of enzymes or lipids differentiate hematopoietic cells, and aids in diagnosis and classification of leukemia
cytochemical staining, Basis of Fab Classification
Leukemic cells are ___% primitive leukemic blast
What are the characteristics of M2-AML
- -resembles M1 except smear shows maturation to or beyond promyeloctes
- *M1 and M2 combined are most common type of AML
- -auer rods are present
In M3-APL leukemic cells are promyelocytes that has what?
- -kidney shaped or bilobed nucleus
- -moderate cytoplasm
- -heavy granulation
- -auer rods common & in multiple
- -negative chemical test
in M3-APL coagulation abnormalities include what?
- -High incidence of DIC-prolonged PT, PTT
- - elevated FDP
- -decreased Factors I and V
Leukemic cells are called what?
M4-AMML are characterized by both what?
monocytic and granulocytic differentiation
this leukemia is positive for periodic acid-Schiff (PAS) stain
What is the size of L2-ALL?
- -larger than L1
What does the nucleus on a L2-ALL look like?
- -irregular in shape
- -clefting & indentation common
- -heterogenous chromatin pattern
- -one or more nucleoli
What does the cytoplasm look like on L2-ALL?
- -variable, often moderately abundant
- -variable cytoplasmic vacuolization
B-cell ALL is also called what?
Burkitt's leukemia or lymphoma
this is asymmetrical enlargement of lymph nodes
B-cell ALL accounts for __-__ % of ALL cases
Name the types of chronic Leukemia
- -chronic lymphocytic leukemia
- -chronic myelogenous leukemia
- -chronic myeloid Leukemia
- -chronic myelocytic leukemia
- -chronic granulocytic leukemia
this has no specific etiologic agent, most common type of leukemia in adults, and twice more common in men
this is 90-95% of cases associated w/ Philadelphia chromosome(conjoing of chromosome 9 and 22, designatedt(9;22)
name the symptoms of CML
- -weight loss-stress sign
- -enlarged spleen
- -night sweats
give the definition of Plasma cell Dirorders
a diverse group of diseases characterized by proliferation of a single clone of cells producing a monoclonal immunoglobin or immunoglobulin fragments
name the etiology of multiple myeloma
- -chemical agents
- *mostly agricultural
- -certain medical conditions
- -not normally consider a myeloproliferative disorder
the some signs and symptoms of mutiple myeloma
- -bone lesions
- *bone pain in 2/3 of cases
- *fatigue SOB
- -GI problems
- *constipation, abdominal pain
this involves IgG or IgA, marked rouleux, elevated ESR, and presence of flames cells in BM
this is over production of IgM, leads to hyperviscosity syndrome, seen in elderly 60-70 years old and equally in both sexes
What are some laboratory features of Waldenstrom's Macroglobulinemia?
- -predominance of prolymphocytes in bone marrow
- -marked rouleaux
this present in neutrophil, and hydrolyzes napthol AS-BI phosphate
What is the principle of MPS?
- -H2O in the presence of peroxidase reacts w/ 3-amino-9-ethylcarbazole
- -blue-black precipitate forms
List the purpose of MPS(mylo cells)
- -differentiate AML or AMol leukemia from ALL
- *positive for AML and AMol
- *negative for ALL
name the principle of SBB
- -lipids are present in granules of Granulocytes and Monocytes
- -SBB is souluble in lipids
- -SBB penetrates granules
- *granules stains black
What are the principle of NSE
- -sodium FLouride inhibits NSE activity in monocytes and macrophages
- -sodium fluoride does not inhibit NSE in other cells
this is absent in monocytes