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Hematology

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Leukorythroblastic reaction

early prematures nucleated RBc and WBC

etiologies

BM infiltraion

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

BM failure

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

spleenectomy

like in SSD

Dx

nucleated RBC

primitive WBC

tear drop RBC

Tumor lysis syn.

post chemo or spontaneus, specially in Borkette's lymphoma

presentaion

Arrythmia

ARF

lytes abnormailties

Ca/Po4. precipitation occur if Ca/PO4 > 60 phosphate rises first Hypocalcemia is next..

prevention

urine ph. keep neutral. acid urine: prevents Ca/Po4 crystals Alkaline urine: prevents uric acid crystals. tx sympt. hypocalcemia. Hydration and Allopurinol.

Neonatal purpura fulminans

sepsis. prot S or C homozygous. need plasma exchange..

Onc

Leukemia

ALL

presentation

Lab

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

CBC

anemia, thrombocytopenia

pancytopenia

normal

WBC

+/- lymphoblasts

normal 1/3. High 1/3. Low 1/3.

smear

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

Organomegaly

2/3.

Bone pain

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

Rehumtoid like dis. responding to steroid

prognosis

high risk. cytometry. Pre B is the most common and best prognosis T cell is more common in older pts.. BM post induction. Age. WBC at diagnosis. polidy: DNA index. Philadelphia chrmosome. B cell: borkette. 2. M2: refractory leukemia. failed to induce remission even after multiple chemo. 3. M3: blasts > 25% after the first induction. clears after multiple induction. < 1 or > 10. > 10000.

treatment

medical. BMT. as last resort after multiple failure. Chemo. CNS chemo. Intrathically: first one is rubinoside then MTX after. Repeat x18 over 2 yrs.. PCP prophlyxis. Induction. 3 weekly cycles ( vincristine x 4Ds, Dounurubicin X 4 ds, Aspargene x 1d) plus daily steroids. Then BM should be cleared.. consolidation. 3-6 ms CNS sanctuary. maintenance. 30 ms. daily MP6 + weekly MTX + monthly vincristine/solumedrol. bactrim. 3 ds/week. Dapsone. if allergic to bactrim.

special types

Leukemia lymphoma syn. agressive T cell leukemia. presentaion. Prognosis. CBC. massive lymphdenopathy and HSM. mediastinal mass. high risk. hg and platelets normal. WBC > 100000. recurrence. testicular recurrence.

AML

also know as ANLL: acute non lymphocytic leukemia

presentaion. dx. special types. Hyperviscosity syn. WBC > 100000 especially in M5. Infiltration. common in type M4 and M5 , the monocyte tumor b/o monocyte existence on tissures. CBC. leukoerythroblastic reaction. DIC. in M3 specially after chemo need pre treatment with Retnoic acid which matures the PreWBC to neurophiles. congenital leukemia. presents in the first month. HSM, infiltration and WBC > 100000. headache. blurry vision. respiratory distress. CNS. skin and bones. usually facial bones. gum hypertrophy. auer bodies. in M1 and 2 and 3 distinguish primitive WBC from sepsis vs AML.

CML

dx. phases. slowly turns into AML. tx. CBC. Low LAP. leukocyte alkaline phosphates LAP: can differentiate between leukocytosis from CML vs sepsis or Leukomoid reaction; latter seen in Down syn,. Incre. B12. BM. philadelphia chromosome. chronic phase. 2 yrs. w/o chemo; 4 yrs with chemo. accelarated phase. Blast crisis. the terminal phase: massive HSM and lymphadenopathy, anemia and thrombocytopenia. BMT. because it&apos;s stem cell disease.. chemo. Neutrophilia. basophilia. thromobytosis. hydroxyurea. Busulfan. interferon.

Burkitt's lymphoma

presentation. Abd mass. Inguinal lymphadenopathy. gerneralized lymphadenopathy in the leukemia/ lymphoma type cervical lymphadenopathy in the african type. Tumor lysis. intessusception.

Lymphoma

presentaion. prognosis. lymphdenopathy. above the diaphragm, in contrast to Burkett&apos;s. specially the supraclavivular node whick is always considered pathologic mostly T cells.. immunodefciency. cyclic fever. labs. Secondary malignancy. ESR. Cu. AML. breast ca.

MDS

pre leukemic

lab. pancytopenia. dimorphic RBC. dec. retic.. large plts.

Solid tumors

Neuroblastoma. wilm's. IVP or CT shows distorion of the kidney calicyles unlike neuroblastoma which showes displacement.. Retinoblastoma. osteogenic sarcoma. Ewing sarcoma. presentaion. prognosis. Prog. diff. presentaion. prog. presentation. presentaion. legs pain. periorbital ecchymoses. Opsoclonus. catecholamines. blasts on peripheral smear. ferritin. < 1 yr. good. associated syn. congenital glucoma. glucoma. photophobia in neonate and tearing.. strabismus and impaired vision. 25 % b/l. secondary tumor: osteogenic sarcoma. painless lesion in distal bone. pathologic fx. high ALphosphatase. painful mass in mid bone. systemic inflammatory. HTN. Diarrhea. spontaneus recovery. aniridia. hemihypertrophy. NF. Beckwith Wiedman syn. 60% at the knee.

coagulation dis.

Von Welliberand. produced from the endothelium connects the plts to the endothelium bleeding immediately carries prot 8. so will results in secondarty low prot VIII superficially bleeding: i.e bleeding into skin and mucosa. 1- clot. plts connected to the endothelium through V.W.F forming plt clot, superficial bleeding. 2- clot. coagulation clot Fibrinogen turned into Fibrin with the enzyme thrombin deep bleeding. thrombophilia. dx. Blood type O have low normal level of VW. tx. plt dysfuncion. low plts. tx. test. quantity. quality. in VWF type 2 which is mainly malfunction of the factor. confirrmation. DDAVP. release v.w.f from endothelium but with limited quantities. good for chronic tx can cause depletion of V.W.F in rare types of the diesease. type 2 b can be given q 48 hrs; to allow regeneration of VWF stores.. EACA. Amicar: aminocabroic acid adjunct to DDAVP in the acute bleeding or for shrot period after surgery, 1 week.. factor replacement. concentrates are contrandicates b/o they have factor 8 but not V.W.F. congenital. acquired. congenital. acquired. factor concentrates. cryoprecipitate. factor 8 only. DDAVP. EACA. Topical thromin. local bleeding, like from a tooth,. prot c. prot s. factor V lyden. VWF titers.. Factor VIII. usually low if VWF is low. because VWF is carrier prot for factor 8. Ristocetin co factor. testing the function of vwf. Plt functin tests. DDAVP stimulation. repeating the tests above before and after DDAVP stimulation. nasal DDAVP. can be given after head trauma prophylactically.. cryopreipitate. FFP. plt aggregation. imapired plt adhesion to V.W.F. Uremia. Myeloproliferative dis. NSAID. auto immune. trapping. dec. production. prot c activity. free total. activated prot C resistance. factor V mutation. Immuno electropheresis. Multimer analysis. prolonged PTT. low factor VIII level. Ristocetin induced plt aggregation. with epinephrine and collagen. this replaced the old fasioned bleeding time but is more reliable.. Glanzman. deficiency plt glycoprotein IIbb/IIIa. Bernard Soulier. ITP. connective tissue dis. immunodeficiency. drugs. neonatal. autoimmune destruction like R.H dis. from different maternal -baby genes. hypersplenism. micro angiopathic dis.. vascular. congenital infection. transient myelodysplasia. mostly in down syn.. BM failure syn. dx. etio. without BM aspiration. with BM aspiration. plt transfusion. stops the bleeding but does not increase the number. splenectomy. for chronic ITP. AIDS. Congenital. Heparin. can evolve into DIC even from heparin for lines patency. DIC. HUS. HUS. Hemangiomas. Kasabach-meritt syn. Fanconi anemia. is insidious , unlike aplastic anemia which is acute. 25% don&apos;t have the dysmorphic features; the triphalangeal thmbs.. T.A.R. thrombocytopenia + absent radius. Wiskott aldrich. T cell immuno deficiency. large plt. impaired adhesion in presen V.W.F. deficiency in plt glycoprot Ib. Winrho. for Rh+ positive only works by overloading the spleen with RBC causing hemolysis so it preserves the plts causes hemoglubinurea from the hemolysis. IVIG. Steroid. 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. Dx. Tx. BM transplant. clinical. insidious; unlike the acute presentation of aplastic anemia 75 % have the charachteristic thumb. lab. Dx: DNA breakage test, clastogenic assay DAB.

anemia

Iron def.. aplastic anemia. SC. thalmsemia. PNH. Auto Hemolysis. tx. Fe: until Ferritin normailze. Reticulocytosis in 3-7 ds Inc. Hg in 2-3 w and normailze in 1 m, avoid tea and soda with meal as it prevents absorption Fe in multivitamin is not sufficient. need 3-6 mg/k and for adult 325 tid.. dx. treat empirically without further tests. if no responce to treatment, consider thalasemia trait and do electropherisi. dx. tx. SCD. trait. minor. major. dx. tx. present. changes into aplastic anemia. lab. tx. acute. lab. clastogenic assay to differentiate from fanconi anemia.. BMT. chemo. Cyclosporine. tx. clinincal. diluted urine. dx. presents like iron deficiency with microcytic anemia but it does not response to Fe. also has inc. reticulocytes unlike Fe anemia Electropherisis: > 3% A2. dx. anemia with massive hepatosplenomaegaly genetic tests. flow cytometry. CD55: decay accelerating factor DAF CD59 homologous restriction factor HRF older tests as sucrose hemolysis and HAM tests are abandoned. steroid. androgen. hemolysis. thrombosis. direct coombs. tests the antibodies attached to the RBC: adds antibodies for the antibodies--> agglutination if pos.. indirect coombs. measures the antibodies in the serum.. warm antibod.. cold antibod.. transfusion. preferred leukofiltered to reduce allergic reaction. exchange transfusion. for acute crisis or chronically post the attack : CVA, chest crisis... hydroxyurea. inc. hg F and so protects from cirisis. aplastic crisis. steroid. immunosuppresion. IVIG. poor tx. acute.