type of granulocyte morphology:

toxic granulation

a hereditary autosomal dominant trait

Lab features:

inclusions will be in monocytes and lymphs

may hegglin inclusions in wbcs- resemble dohle bodies

thrombocytopenia with giant platelets

abnormal bizzare appearing platelets

inherited as an autosomal reccessive trait

associated with disorders that prevent normal breakdown of mucopolysaccharides

granulocytes, lymphs, and monocytes have heavy azurophilic granulation

characterized by presence of prominent, dark staining, coarse cytoplasmi granules that: are made of mucopolysacchrde deposits; may totally obscure the nucleus of cells

granules resemble toxic granulation except: larger, permanent feature of the cell,present even without infection

autosomal recessive trait caused  by lysosomal defect

giant lysosomal granules produced in wbcs: cells cannnot release their contents after bacteria digestion

neutrophils shows giant grey-green cytoplasmic granules

lymphocytes and monocytes show single red granule in cytoplasm

autosomal dominant trait

results  in abnormal maturation in neutrophils  neucleus

appears to function normally

decreased segmentation of the nucleus

nucclear chromatin coarse and condensed

majority of the neutrophils have bilobed nucleus that is: dumbell shaped has pince-nez appearance ( two symetrical lobes)

inherited primarily as a rare sex-linked recessive trait

seen primarily in males

generally fatal during early childhood due to recurring bacterial infections

wbc unable to kill catalase-positive organisms: catalase neutralizes hydrogen peroxide; hydorgen peroxide assists in oxygen- dependant bactericidal

normal wbc morphology

number of wbcs increase during infection

granulomas ( granular tumor or growth) in many organs: site of chronic infection

results from deficiency of the enzyme beta-glucocerebrosidase

deficeincy leads to accumulation of glucocerebrosides mosty in monocytes/ macrophages; called gaucher cells

characterized by severe bone pain if bone is involved

cbc: leukopenia, thrombocytopenia

hepatosplenomegaly (simultaneous enlargement of both spleen and liver)

bone marrow shows gaucher cells: cells look like unfolded crumpled piece of pape

increased serum acid phosphatase

result of dificiency of the enzyme sphingomyelinase

leads to accumulation of sphingomyelinase in histocytes

thrombocytopenia

presence of sea-blue histocytes in bone marrow: diagnostic of the syndrome

cel stain a striking intense blue using wright's stain or may-grunwald stain

macrophages have globular cytoplasm ( a cytoplasmic inclusion)

reault of dificenciy of the enzyme hexaminosidase

enzyme dificency tested prenatally

leads to accumlation of sphingolipids in lymphocytes

central nervous system affected mostly

vacuolated lymphocytes inperipheral blood

vacuolated histocytes (foam cells) in bone marrow

a bening illness caused by epstein barr virus (ebv)

virus enters body orally throught the lymphoid tissues in the pharnyx; infects b-lymphocytes; runs coarse and complete recovery usually occurs in 2 months; primarily in children young adults all races

fatigue

sore throat

dysphagia: difficulty swallowing

general malaise

enlargement of the lymph nodes

splenomegaly and hepatomegaly

diagnostic lab features include: presence of atypical lymph; positive monospot test

cbc: normal rbc and plt counts; slightly increased wbc count (can be up to 20,000/cu.mm

differential: absolute increase in lymphs (20-90% atypical lymph)

liver enzyme test may be abnormal

a neutrophilic respose to bacterial infection marked by: accelerated relase of neutrophils; accompanied by shift to the left

increased wbc count

neutrophillia with morphological sings of infection ( shift to the left): presence of metamyelocytes; increased number of bands

wbc anomalies: toxic granulation; dohle bodies; cytoplasmic vacuolization

10-100 x 10/mm3

no esos, basos, or philidelphia chromosome

LAP increased

round oval light blue staining area found in the cytoplasm

remnanats of dna

seen with toxic granulation in infections and burns

slender rod-shaped bodies within the cytoplasm that are similar to azurophilic granules

seen within the cytoplasm of myeoblasts in acute myeoblastic leukemia

round clear spaces within granulocyte cytoplasm

seen in patients with severe bacterial infections

usually  colonal

all abnormal cells and appear very similar to one another

seen in cancer

lymph with abundant cytoplasmm often indented by surrounding rbcs

often seen in infectious mononucleosis

blue cytoplasm eccentric nucleus promininent nuclear clearing

rarely seen except in cases of severe inflammation or end stage of mutiple myeloma

damaged fragile wbcs with a frayed nucleus and no cytoplasmic material

if many, mainly associated with chronic lymphoblastic leukemia

ratio of the # of mature and immature granulocytes to # of mature and immature rbcs

normal range 2:1- 4:1

exposure to ionizing radiation

chemical agents assoc with leukemia include: benzene and cytotoxic alkylating agents; alkylating agents used to treat neoplasm

viruses: epstein-barr virus; human t-cell lymphotrophic virus

genetic factors: not inherited; clusters in families; increased in persons with congential chromosomal abnormalities as in down's syndrome

age: clinically important factor in diagnosis; different leukemias affect different age groups

race: usually more common in whites than blacks

leukemic cells: immature ( blast and pro stage)

thrombocytopenia: mild to severe

wbc count: usually elevated; some patients may have normal to decreased counts

affects all ages

if untreated less than  6mths course time

onset is sudden, rapidly

leukemic cells- mature

thrombocytopenia: mild

wbc count: increased

affects adults

course 2-6 yrs

symptoms of long duration

stains alkalike phosphatase present in the neutrophil

helpfil in differientaiting chronic myelogenous leukemia from leukemoid reaction or polycythemia vera

stains peroxidase present in primary granules of myeloid cells; demonstrates presence of auer rods

Positive- acute myelogenous or monocytic leukemias

negative- acute lymphocytic leukemia

leukemia: abnormal cells are present in both bone marrow and peripheral blood

lymphoma: abnormal growth of cells is confined to the lymphatic tissue

stains high molecular weight carbohydrates normally present in most blood cells types except pronormoblasts

helps diagnosis erythroleukemia

stains phospholipids and lipoproteins present in granules of monocytes and granulocytes

Positive- acute myelogenous and myelomonocytic leukemias

negative- acute lyphocytic leukemia

stains acid phosphatase present in lysosomes of all hematopoietic cells

identifies t-cell acute lymphocytic leukemia

acid phophatase present in all hematopoietic cells

located in lysosomes 

helpful in diagnosing hairy cell leukemia

stains esterases in monocytes and macrophages

negative- granulocytic leukemias

positive- monocytic leukemias

stains esterases present in mature and immature neutrophils and mast cels

positive- neutrophilic cells

negative- monocytic cells

malignant: clonal disease

characterized by the rapid proliferation of abnormal cells that: accumulate in the bone marrow; interfere with the production of normal blood cells

most common acute leukemia affecting adults

cbc: blood count variables

blasts present in peripheral smear

auer rods present in: myeoblasts, monoblasts, promyelocytes

nrbcs may be seen

psuedo pelger-huet cells

increased m:e ration

charaterized by replacement of normal hematopoietic elements in the bone marrow by abnormal lymphoid cells

results in decreased rbcs, phagocytes, and plts

most common malignant disease in children

wbc count elevated in 60-70% of patients- blast forms predominate

close to 100% lymphoblasts and lymphocytes seen on peripheral smear

clonal stem cell disorder affecting the: monocytic, erythocytic, and megakaryocytic stem lines

most causes (95%) unknown

philidelphia chromosome formation underlying common finding : formation occurs when chromosome 9 and 22 conjoin; present in 70-90% of cml patients

accounts for 20-25% of all leukemia cases in western countries

disease marked by overwhelming accumulation of myeloid cells within the bone marrow and peripheral blood

cell counts marked by: extremely increased wbc counts

possible increase plt counts

peripheral smear shows: presence of neutrophils in all stages of development; increased basos and eos; increased plts; nrbcs

hypercellular

m:e ratio generally at least 10:1; normal is 3:1

low to absent lap

most cases clonal proliferation of b-lymph: 98% b-cell; 2% t-cell

causes: none; possibal viral cause

two times more common in men

most common type of leukemia in adults over 50

normocytic/normochromic anemia with slight dec in plts

increased wbc ct: 20-200 x 10^9/L

peripheral smear: often see smudge cells; usually shows 60-95% lymphocytes

myeloproliferative disorder:

chronic monocytic leukemia

cells negative for philidelphia chromosome: manifest with peripheral blood, bone marrow, and extramedullary tissues

usually the crisis is acute myeloid or acute lymphoblastic in nature

symptoms: severe anemia (usually n/n)

leukocytopenia

thrombocytopenia

myeloproliferative disorder:

polycythemia vera

characterized by an accelerated production of rbcs

stimulates excess production of myeloids and megakaryocytes

definitive key to diagnosis: increase in absolute rbcs

usually seen in patients older than 60

 increased hct, hgb, and red cell mass

increased wbc ct: 80% of the cases

increased plt ct: 50% of the cases

morph: nrbcs often present; poikilocytosis

myeloproliferative disorder:

essential thrombocythemia

clonal proliferation of megakaryocytes in the bone marrow

megakaryocytes larger than normal and may be dysplastic in appearance

plts:marked thrombocytosis; giant plts; microthrombocytes; plt aggregates; abnormally granulated plts; megakaryocytic cytoplasmic fragments; abnormal plt function

rbcs: n/n anemia

hjb, pappenheimer bodies, target cells, acantho

stem cel defect resulting in an abnormal proliferation of granulocytes, rbcs, and megakaryocytes

may transform into acute leukemia

increased wbc ct in 50% of patients

morph: nrbcs, tear drop, immature neutrophils, dwarf megakaryocytes often present, along with large bizarre plts

medium sized lymphs (15-30 um in diameter)

nucleus:round to oval with fine chromatin; 1 to 5 distinct nucleoli

abundant cytoplasm with hair-like or ruffled projections 

larger than normal lymphs

scant cytoplasm

cerebriform nucleus: with or without nucleoli; with fine chromatin

presence of reed-sternburg cells during lymph node biopsy

cells look like large, multinucleated cells resembling an owl's eye

cells react to the neoplasm rather than the neoplastic cells

moderately increased wbc count with moncytosis neutrophilia and variable eosinophilia

toxic granulation

large bizzare plts

proliferation of malignant lymphocytes arrested at certain stages of maturation

abnormal lympocytes may be present on the peripheral smear

result from abnormal proliferation of b-lymphocytes

plasma cells grow out of control and form a myeloma usually in the bone marrow

may lead to plasma cell leukemia

highest risk in black men over 80

increased esr

bence-jones protein present in urine

peripheral blood smear- marked rouleax

bone marrow estimation reveals abnormal plasma cells

see chart pg 135-136

non-hodgkins lymphoma produces large amounts of an abnormal protein called macroglobulin

lymphoma cells grow mainly in teh bone marrow, where they crowd out the normal cells that make different blood cells

n/n anemia

marked rouleaux formation on peripheral blood smear

abnormal bleeding: prolonged bleeding time: PT, PTT; predominance of prolymphocytes in bone marrow

headaches, dizziness, and visual impairment

increased blood pressure

splenomegaly- 75% of the cases

hepatomegaly- 35% of the cases

phlebitis

erythromelagia- painful red extremities

bone lesions

anemia

GI

fatigue/ weight loss

enlargement of lymph nodes, liver and spleen

neurobological changes

congestive heart failure

extensive bruising called cryoglobulinemic purpura