Categorii: Tot - anemia

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Hematology

Hematology involves the study of blood and its disorders, focusing on issues related to coagulation and thrombophilia, which can lead to abnormal clotting. Diagnostic tests for these conditions include measurements of protein C and S activity, factor V mutation, and activated protein C resistance.

Hematology

Hematology

  • 1:48

    • anemia

    Auto Hemolysis

    cold antibod.

    poor tx

    warm antibod.

    immunosuppresion

    indirect coombs

  • measures the antibodies in the serum.

    • direct coombs

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

    • PNH
    present

  • changes into aplastic anemia

    • thrombosis

    hemolysis

    androgen

    steroid

    flow cytometry

  • CD55: decay accelerating factor DAF
  • CD59 homologous restriction factor HRF
  • older tests as sucrose hemolysis and HAM tests are abandoned

    • thalmsemia
    major

  • anemia with massive hepatosplenomaegaly
  • genetic tests

    • minor

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

    • SC
    trait

    diluted urine

    SCD

    clinincal

    aplastic crisis

  • inc. hg F and so protects from cirisis

    • exchange transfusion

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

    • transfusion

  • preferred leukofiltered to reduce allergic reaction

    • aplastic anemia

  • Cyclosporine

  • clastogenic assay to differentiate from fanconi anemia.

    • acute

    Iron def.

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

  • 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.

    • coagulation dis.

    thrombophilia
    test

    factor V lyden

    factor V mutation

    activated prot C resistance

    prot s

    free total

    prot c

    prot c activity

    2- clot

  • coagulation clot
  • Fibrinogen turned into Fibrin with the enzyme thrombin
  • deep bleeding

    • Topical thromin

  • local bleeding, like from a tooth,

    • cryoprecipitate

  • factor 8 only

    • factor concentrates

    1- clot

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

    • low plts

    dec. production

    BM failure syn

    Wiskott aldrich

  • T cell immuno deficiency

    • T.A.R

  • thrombocytopenia + absent radius

    • Fanconi anemia

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

    • Tx

  • BM transplant

  • Dx: DNA breakage test, clastogenic assay DAB

    • clinical

  • insidious; unlike the acute presentation of aplastic anemia
  • 75 % have the charachteristic thumb

    • transient myelodysplasia

  • mostly in down syn.

    • congenital infection

    trapping

    vascular

    Hemangiomas

  • Kasabach-meritt syn

    • micro angiopathic dis.

    HUS

    hypersplenism

    auto immune

    neonatal

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

    • drugs

    Heparin

  • can evolve into DIC
  • even from heparin for lines patency

    • immunodeficiency

    Congenital

    AIDS

    connective tissue dis

    ITP

    splenectomy

  • for chronic ITP

    • plt transfusion

  • stops the bleeding but does not increase the number

    • with BM aspiration

    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

    • without BM aspiration

    IVIG

    Winrho

  • for Rh+ positive only
  • works by overloading the spleen with RBC causing hemolysis so it preserves the plts
  • causes hemoglubinurea from the hemolysis

    • plt dysfuncion

    acquired

    NSAID

    Myeloproliferative dis

    Uremia

    congenital

    imapired plt adhesion to V.W.F

    Bernard Soulier


    etio

    deficiency in plt glycoprot Ib

    impaired adhesion in presen V.W.F

    large plt

    plt aggregation

    Glanzman

  • deficiency plt glycoprotein IIbb/IIIa

    • 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

    • factor replacement

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

    • FFP

    cryopreipitate

    EACA

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

    • 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.

    • nasal DDAVP

  • can be given after head trauma prophylactically.

  • Blood type O have low normal level of VW

    • confirrmation

    DDAVP stimulation

  • repeating the tests above before and after DDAVP stimulation

    • quality

  • in VWF type 2 which is mainly malfunction of the factor

    • Plt functin tests

    with epinephrine and collagen

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

    • Ristocetin induced plt aggregation

    Ristocetin co factor

  • testing the function of vwf

    • quantity

    Factor VIII

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

    • low factor VIII level

    prolonged PTT

    VWF titers.

    Multimer analysis

    Immuno electropheresis

    Onc

    Solid tumors
    Ewing sarcoma

    systemic inflammatory

    painful mass in mid bone

    osteogenic sarcoma

    high ALphosphatase

    pathologic fx

    painless lesion in distal bone

    60% at the knee

    Retinoblastoma

    prog

    secondary tumor: osteogenic sarcoma

    25 % b/l

    strabismus and impaired vision

    glucoma

  • photophobia in neonate and tearing.

    • diff

    congenital glucoma

    wilm's

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

    • Prog

    associated syn

    Beckwith Wiedman syn

    NF

    hemihypertrophy

    aniridia

    Neuroblastoma

    < 1 yr. good

    spontaneus recovery

    ferritin

    blasts on peripheral smear

    catecholamines

    Diarrhea

    HTN

    Opsoclonus

    periorbital ecchymoses

    legs pain

    MDS

  • pre leukemic

    • lab

    large plts

    dec. retic.

    dimorphic RBC

    Lymphoma

    Secondary malignancy

    breast ca

    labs

    Cu

    ESR

    cyclic fever

    immunodefciency

    lymphdenopathy

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

    • Leukemia
    Burkitt's lymphoma

    Tumor lysis

    Inguinal lymphadenopathy

  • gerneralized lymphadenopathy in the leukemia/ lymphoma type
  • cervical lymphadenopathy in the african type

    • Abd mass

    intessusception

    CML

    tx

    chemo

    interferon

    Busulfan

    hydroxyurea

  • because it's stem cell disease.

    • phases

  • slowly turns into AML

    • Blast crisis

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

    • accelarated phase

    chronic phase

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

    • BM

  • philadelphia chromosome

    • Incre. B12

    Low LAP

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

    • thromobytosis

    basophilia

    Neutrophilia

    AML

  • also know as ANLL: acute non lymphocytic leukemia

    • congenital leukemia

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

    • dx

    DIC

  • in M3 specially after chemo
  • need pre treatment with Retnoic acid which matures the PreWBC to neurophiles

    • leukoerythroblastic reaction

    auer bodies

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

    • Infiltration

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

    • gum hypertrophy

    skin and bones

  • usually facial bones

    • CNS

    Hyperviscosity syn

  • WBC > 100000
  • especially in M5

    • respiratory distress

    blurry vision

    headache

    ALL

    special types

    Leukemia lymphoma syn

    agressive T cell leukemia

    Prognosis

    recurrence

    testicular recurrence

    mediastinal mass

    massive lymphdenopathy and HSM

    WBC > 100000

    hg and platelets normal

    treatment

    BMT

  • as last resort after multiple failure

    • medical

    PCP prophlyxis

    Dapsone

  • if allergic to bactrim

    • bactrim

  • 3 ds/week

    • CNS chemo

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

    • Chemo

    maintenance

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

    • consolidation

  • 3-6 ms
  • CNS sanctuary

    • Induction

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

    • prognosis

    high risk

    WBC at diagnosis

    > 10000

    Age

    < 1 or > 10

    BM post induction

    3

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

    2

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

    cytometry

  • Pre B is the most common and best prognosis
  • T cell is more common in older pts.

    • B cell: borkette

    Philadelphia chrmosome

    polidy: DNA index

    presentation

    Rehumtoid like dis. responding to steroid

    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

    • Organomegaly

    2/3

    Lab

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

    • smear

    blasts

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

    • CBC

    WBC

  • +/- lymphoblasts

    • Low 1/3

    High 1/3

    normal 1/3

    normal

    pancytopenia

    anemia, thrombocytopenia

    others

    Neonatal purpura fulminans
    prot S or C homozygous

  • need plasma exchange.

    • sepsis
    Tumor lysis syn.

  • post chemo or spontaneus, specially in Borkette's lymphoma

    • prevention

    Hydration and Allopurinol

    tx sympt. hypocalcemia

    urine ph

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

    • presentaion

    lytes abnormailties

    Ca/Po4

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

    • ARF

    Arrythmia

    Leukorythroblastic reaction

    early prematures nucleated RBc and WBC

    Dx

    tear drop RBC

    primitive WBC

    nucleated RBC

    etiologies

    spleenectomy

  • like in SSD

    • BM failure

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

    BM infiltraion

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

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