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What is the def. of WBC anomalies?
congenital or hereditary condition where the WBC's deviate from the normal standard
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what is the Etiology of Inherited (true) Pelger-huet?
- -autosomal dominant
- -abnormal maturation of nucleus
- -appear to function normally
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What are the laboratory features of an Inherited (true) Pelger-huet?
- -decreased segmentation of nucleus
- -coarse and condense chromatin
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this cell maybe drug induced, is secondary to leukemia(CmL) and is 10% normal 3 lobed nucleus
Pseudo-Pelger-Huet
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this cell is shift to the left, metamyelocytes and bands are present and is a response to severe infection
Pelger-Huet
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this is a defect in the lysomes and it leads to delayed degranulation(stained blue-purple granules)
chediak-higashi
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this cell is autosomal dominant
May-Hegglin
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What are the laboratory features of a may-hegglin
- -dohle bodies(blue straining area made up of rough plasma eticular & RNA)
- -thrombocytopenia
- -giant, bizarre platelets
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this cell is rare sex-linked recessive, fatal due to infetion, and defect in WBC; unable to kill catalase positive organism
CGD
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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
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this disease is the deficiency of enzyme beta-glucocerebroside
Gausher's diesease
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what are the laboratory findings of Gaucher's disease
- -leukopenia
- -thrombocytopenia
- -hepatosplenomegaly
- -in bone marrow looks like unfolded crumpled paper
- -increased serum acid phosphatase
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what is the etiology of tay-sachs disease?
- -autosomal recessive
- -deficiency oof hexaminosidase
- -seen in mostly jews
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what is hexaminosidase?
accumulation of sphingolipids in lymphocytes
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this disease death will occur within a few a years, and is a major site of pathology is central nervous system
Tay-sachs disease
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what is the etiology of a neiman-pick?
- -aka Sea-blue histiocytes
- -due to defiency in sphigomyelinase
- (leads to accumulation of sphigomyelin in histocytes)
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this the deficiency in enzymes that break down mucopolysaccharide
Hunter's & Hurler's
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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
infectious monocucleosis
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What are the symptoms of infectious monocucleosis?
- -general malaise
- -enlarge lymph nodes
- -splenomegaly
- -hepatomegaly
- -benign illness
-
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what is the etiology of Leukemoid reaction?
- -neutrophilic response to infection
- -accelerated release of neutrophil
- -mimics early stage of leukemia
- (hence leukemoid reaction)
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this has dark blue-black granules seen in severe infection (toxic granulation)
leukemoid reaction
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explain the Leukomoid reaction vs. CML
- WBC count---10-100---30-500
- Eosinophils---absent---present
- Basophils---absent---present
- Philadelphia chromosome---absent---present
- LAP---inceased---decreased
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this is the malignat disease of hematopoietic tissue
Leukemias
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this is normal bone marrow elements replaced by neoplastic cells, bone marrow is always involved, and infiltates spleen, liver, lymph nodes
leukemias
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explain the acute vs. chronic
- ------------acute------chronic
- age:------all ages----adults
- onset:----sudden-----insidous
- cells:-----immature(blast)----mature
- anemia--mild to severe--mild
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this will become mild-severe when acute and mild when chronic
Thrombocytopenia
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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
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what is the purpose of FAB classification
to attain better discrimination in therapy and prognosis
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what are the basis of FAB classification
- -WBC morphology
- -cytochemical staining
- -immunologic marker studies
- -cytogenetics
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explain the basis of FaB classification
- wbc morphology:
- -----------AML Blast vs ALL Blast
- -size-----large-----------small
- -cytoplasm:moderate--scant
- -chromatin:fine,lace---dense
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this is prominent AML blast usually >2
nucleoli
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this indistinct ALL Blast usually 2 or less
nucleoli
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this is present in 50-60% in AML Blast
Auer Rods
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this is never present in ALL Blast
Auer Rods
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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
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Leukemic cells are ___% primitive leukemic blast
100%
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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
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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
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in M3-APL coagulation abnormalities include what?
- -High incidence of DIC
- -prolonged PT, PTT
- - elevated FDP
- -decreased Factors I and V
- -thrombocytopenia
-
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Leukemic cells are called what?
myelomonocytic cells
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M4-AMML are characterized by both what?
monocytic and granulocytic differentiation
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this leukemia is positive for periodic acid-Schiff (PAS) stain
M6-AEL
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What is the size of L2-ALL?
- -larger than L1
- -heterogenous
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What does the nucleus on a L2-ALL look like?
- -irregular in shape
- -clefting & indentation common
- -heterogenous chromatin pattern
- -one or more nucleoli
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What does the cytoplasm look like on L2-ALL?
- -variable, often moderately abundant
- -variable cytoplasmic vacuolization
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B-cell ALL is also called what?
Burkitt's leukemia or lymphoma
-
this is asymmetrical enlargement of lymph nodes
lymphoma
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B-cell ALL accounts for __-__ % of ALL cases
2-5%
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Name the types of chronic Leukemia
- -chronic lymphocytic leukemia
- -chronic myelogenous leukemia
- -chronic myeloid Leukemia
- -chronic myelocytic leukemia
- -chronic granulocytic leukemia
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this has no specific etiologic agent, most common type of leukemia in adults, and twice more common in men
CLL
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this is 90-95% of cases associated w/ Philadelphia chromosome(conjoing of chromosome 9 and 22, designatedt(9;22)
CML
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name the symptoms of CML
- -weight loss-stress sign
- -enlarged spleen
- -fever
- -night sweats
- -malaise
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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
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name the etiology of multiple myeloma
- -radiation
- -chemical agents
- *mostly agricultural
- -certain medical conditions
- -not normally consider a myeloproliferative disorder
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the some signs and symptoms of mutiple myeloma
- -bone lesions
- *bone pain in 2/3 of cases
- -anemia
- *fatigue SOB
- -GI problems
- *constipation, abdominal pain
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this involves IgG or IgA, marked rouleux, elevated ESR, and presence of flames cells in BM
Multiple Myeloma
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this is over production of IgM, leads to hyperviscosity syndrome, seen in elderly 60-70 years old and equally in both sexes
Waldenstrom's Macroglobulinemia
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What are some laboratory features of Waldenstrom's Macroglobulinemia?
- -predominance of prolymphocytes in bone marrow
- -marked rouleaux
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this present in neutrophil, and hydrolyzes napthol AS-BI phosphate
LAP
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What is the principle of MPS?
- -H2O in the presence of peroxidase reacts w/ 3-amino-9-ethylcarbazole
- -blue-black precipitate forms
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List the purpose of MPS(mylo cells)
- -differentiate AML or AMol leukemia from ALL
- *positive for AML and AMol
- *negative for ALL
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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
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What are the principle of NSE
- -sodium FLouride inhibits NSE activity in monocytes and macrophages
- -sodium fluoride does not inhibit NSE in other cells
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this is absent in monocytes
SE(specific esterase)
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