Hematology

early prematures nucleated RBc and WBC

by turmors so it releases early cell linescan see teardrop RBC as well

RBC then will be formed in other organs like spleen and liver such as in thelasemia.

like in SSD

post chemo or spontaneus, specially in Borkette's lymphoma

precipitation occur if Ca/PO4 > 60phosphate rises firstHypocalcemia is next.

keep neutral.acid urine: prevents Ca/Po4 crystalsAlkaline urine: prevents uric acid crystals

need plasma exchange.

initial w/u should include CBC, LDH, Uric acid and ESR

+/- lymphoblasts

atypical lymphocytes can mimic blasts: seen in viral infectionsneed manual diff and smear to differentiate both

can mimic grwoth pain and responds well to steroids as well; however this results in selecting steroids resistant leukemic cellson xray :new leukemic lines over the metaphisis

Pre B is the most common and best prognosisT cell is more common in older pts.

M2: refractory leukemia. failed to induce remission even after multiple chemo

M3: blasts > 25% after the first induction. clears after multiple induction

3 weekly cycles ( vincristine x 4Ds, Dounurubicin X 4 ds, Aspargene x 1d) plus daily steroids.Then BM should be cleared.

3-6 msCNS sanctuary

30 ms. daily MP6 + weekly MTX + monthly vincristine/solumedrol

Intrathically: first one is rubinoside then MTX after. Repeat x18 over 2 yrs.

3 ds/week

if allergic to bactrim

as last resort after multiple failure

agressive T cell leukemia

testicular recurrence

also know as ANLL: acute non lymphocytic leukemia

WBC > 100000especially in M5

common in type M4 and M5 , the monocyte tumor b/o monocyte existence on tissures

usually facial bones

in M1 and 2 and 3distinguish primitive WBC from sepsis vs AML

in M3 specially after chemoneed pre treatment with Retnoic acid which matures the PreWBC to neurophiles

presents in the first month. HSM, infiltration and WBC > 100000

leukocyte alkaline phosphates LAP: can differentiate between leukocytosis from CML vs sepsis or Leukomoid reaction; latter seen in Down syn,

philadelphia chromosome

slowly turns into AML

2 yrs. w/o chemo; 4 yrs with chemo

the terminal phase: massive HSM and lymphadenopathy, anemia and thrombocytopenia

because it's stem cell disease.

gerneralized lymphadenopathy in the leukemia/ lymphoma typecervical lymphadenopathy in the african type

above the diaphragm, in contrast to Burkett's. specially the supraclavivular node whick is always considered pathologic mostly T cells.

pre leukemic

IVP or CT shows distorion of the kidney calicyles unlike neuroblastoma which showes displacement.

photophobia in neonate and tearing.

produced from the endotheliumconnects the plts to the endotheliumbleeding immediatelycarries prot 8. so will results in secondarty low prot VIIIsuperficially bleeding: i.e bleeding into skin and mucosa

Blood type O have low normal level of VW

usually low if VWF is low. because VWF is carrier prot for factor 8

in VWF type 2 which is mainly malfunction of the factor

testing the function of vwf

this replaced the old fasioned bleeding time but is more reliable.

repeating the tests above before and after DDAVP stimulation

release v.w.f from endothelium but with limited quantities.good for chronic txcan cause depletion of V.W.F in rare types of the diesease. type 2 bcan be given q 48 hrs; to allow regeneration of VWF stores.

can be given after head trauma prophylactically.

Amicar: aminocabroic acid adjunct to DDAVP in the acute bleeding or for shrot period after surgery, 1 week.

concentrates are contrandicates b/o they have factor 8 but not V.W.F

plts connected to the endothelium through V.W.F forming plt clot,superficial bleeding

deficiency plt glycoprotein IIbb/IIIa

for Rh+ positive onlyworks by overloading the spleen with RBC causing hemolysis so it preserves the pltscauses hemoglubinurea from the hemolysis

steroid is contradicted without BM aspiration because if it was due to leukemia it causes partial treatment and subsequent resistence.the effect is slower than IvIG

stops the bleeding but does not increase the number

for chronic ITP

can evolve into DICeven from heparin for lines patency

autoimmune destruction like R.H dis. from different maternal -baby genes

Kasabach-meritt syn

mostly in down syn.

is insidious , unlike aplastic anemia which is acute.25% don't have the dysmorphic features; the triphalangeal thmbs.

insidious; unlike the acute presentation of aplastic anemia75 % have the charachteristic thumb

Dx: DNA breakage test, clastogenic assay DAB

BM transplant

thrombocytopenia + absent radius

T cell immuno deficiency

coagulation clotFibrinogen turned into Fibrin with the enzyme thrombindeep bleeding

factor 8 only

local bleeding, like from a tooth,

Fe: until Ferritin normailze.Reticulocytosis in 3-7 dsInc. Hg in 2-3 w and normailze in 1 m,avoid tea and soda with meal as it prevents absorptionFe in multivitamin is not sufficient. need 3-6 mg/k and for adult 325 tid.

treat empirically without further tests.if no responce to treatment, consider thalasemia trait and do electropherisi

clastogenic assay to differentiate from fanconi anemia.

Cyclosporine

preferred leukofiltered to reduce allergic reaction

for acute crisis or chronically post the attack : CVA, chest crisis..

inc. hg F and so protects from cirisis

presents like iron deficiency with microcytic anemia but it does not response to Fe. also has inc. reticulocytes unlike Fe anemiaElectropherisis: > 3% A2

anemia with massive hepatosplenomaegalygenetic tests

CD55: decay accelerating factor DAFCD59 homologous restriction factor HRFolder tests as sucrose hemolysis and HAM tests are abandoned

changes into aplastic anemia

tests the antibodies attached to the RBC: adds antibodies for the antibodies--> agglutination if pos.

measures the antibodies in the serum.