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PANCE > Hematology - 5% > Flashcards

Hematology - 5% Flashcards

1
Q

Acute Lymphocytic Leukemia (ALL)

A

MC in children 3-7yo and MCC childhood cancer

highly responsive to chemotherapy

Sxs

  • lymphadenopathy
  • Bone pain
  • bleeding from nose and gums, diffuse ecchymosis

Dx:

  • bone marrow > 20% blasts

Tx

  • chemotx - Imatinib (+philly) or methotrexate (CNS dz)
  • relapse = transplant
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2
Q

Acute Myeloid Leukemia (AML)

A

MC Adults >50yos

RF toxin exposure - benzene, radiation (organic chemist)

Sxs:

  • gingival hyperplasia, bone pain, pallor, bleeding

Dx:

  • bone marrow with Auer Rods
  • >20% blasts + Pancytopenia

Tx

  • Induction chemo (short) => transplant, better prognosis if <60yo
  • Tumor lysis syndrome - 48-72 hrs after tx, incr in uric, K, phostphate and decr in Ca –> treat with allopurinol and IV fluids
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3
Q

Anemia of Chronic Dz

A

Eti - older patients w/ inflammation, cancer, RF, liver

MCC - chronic renal failure - impaired EPO production

Dx:

  • Normal or microcytic MCV
  • low TIBC and Fe
  • High Ferritin stores (nl to 3x)
  • low Serum EPO

Tx

  • IV EPO
  • Tx underlying dz
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4
Q

Antiphospholipid Antibody Syndrome

Hypercoaguable State

A

Autoimmune, often a/w SLE

AutoABs attack platelet membranes, activates endothelial cells + plts - breaks down platelets

Sxs

  • thromboses and recurrent spont abortions

Dx:

  • lupus anticoag
  • prolonged Dilute Russell viper Venom Time (DRVVT)
  • Prolonged PTT
  • anti-B2 glycoprotein
  • anticardiolipin

Tx

  • high dose IV hep w/ thrombic event, PO Anticoag indef
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5
Q

Antithrombin III Deficiency

Hypercoaguable State

A

Recurrent venous thrombosis and PE, Repetitive IU fetal death

AT III - is a natural anticoag; inhibits thrombin (IIa), and Xa, and other proteases

  • a/w VTE - 1st ep during 20-30yo

Tx

  • asymptomatic - anticoag before sx
  • w/ acute thrombotic event = high dose IV Hep, PO Anticaog indefinitely
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6
Q

Aplastic Anemia

A

All three cell lines decreased - LOW WBC, RBC, Plts Pancytopenia

eti - chemicals, drugs, radiation (ACE-I, gold, sulfa, phenytoin, chemo, XRT)

Sxs:

  • severe pallor, weakness
  • petechiae
  • ecchymosis
  • mucosal bleeding
  • severe infx

Dx:

  • pancytopenia
  • BM bx - gold

Tx - stop causative agent, RBC transfusion, BMT

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7
Q

Chronic Lymphocytic Leukemia (CLL)

A

MCC leukemia in adults; esp B cell type

Middle age pt - peak at 50yo

asymptomatic - incidental finding

Sxs:

  • recurrent infections*** - pna, renal infx,
  • splenomegaly, lympadenopathy

Dx:

  • Smudge cells - lymphocytosis - on perip smear, mature lymphocytes

Tx:

  • chemo or allogenic stem cells - cure
  • younger = more aggressive in patients
  • Fludarabine - IV 5d/wk x4-6 mo = not cure
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8
Q

Chronic Myelogenous Leukemia (CML)

A

>50yo, AA descent has poorer prognosis

Sxs:

  • Usu asymptomatic until blast crisis (acute leukemia)
  • fever, nt swts, anorexia (gradually)
  • Hyperuricemic

Dx:

  • Incr LDH, uric acid
  • WBC > 150,000 in the setting of + Philadelphia chromosome
  • left myeloid shift on perip smear

Tx

  • Imatinib/Gleevac - Tyrosine kinase inhibitor = not cure
  • BM or stem cell transplant - better prognosis if younger
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9
Q

Disseminated Intravascular Coagulation

Thrombocytopenia

A

Abnormal activation of coagulation sequence

  • microthrombi throughout microcirculation
  • consumption of plts, fibrin, coag factors
  • Bleeding and thrombosis occurs simultaneously
  • Burn, trauma, OBGYN patients

Dx:

  • ↓ Platelets,
  • ↑ bleeding time, ↑ PT, ↑ PTT
    • D-Dimer (fibrin degradation product)

Tx:

  • Supportive
  • cryoprecipitate, FFP
  • plt transfusion if < 30,000
  • Heparin
  • tx cause
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10
Q

Factor V Leiden

Hypercoaguable State

A

MCC hypercoaguable state, Autosomal dominant

Mutated factor V resistant to breakdown by Protein C - amplifies production of thrombin causing more clot formation

  • increased risk of DVT and PE in young pts

Dx

  • activated protein C resistance assay - if +, confirm with DNA testing for factor V Leiden
  • normal PT/PTT

Tx

  • LMWH bridge to warfarin
  • long term antithrombic tx not rec’d
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11
Q

Hemolytic Anemia - G6PD

A

hemolytic anemia - premature breakdown of RBCs,

AA and Mediterranean; Autosomal Recessives

Oxidant sensitivity - after infection or medication causing oxidative stress

  • Fava beans
  • sulfonamides
  • antimalarials
  • analgesics - phenacetine - acetylsalicyclic acid

Dx - Bite cells, Heinz body** on smear

Tx - avoid drugs,

acute - blood transfusion

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12
Q

Hemolytic Uremic Syndrome (HUS)

Thrombocytopenia

A

Low platelets + anemia + renal failure (a/w E.coli O157:H7 and diarrheal illness in a child

post infection - E coli or shigella

Children

Severe kidney problems

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13
Q

Hemophilias A and B

Clotting Factors Disorder

A

hereditary bleeding disorder - two forms

  • Hemophilia A - Factor VIII/8 Deficiency - More common
  • Hemophilia B - Factor IX/9 Deficiency (Christmas Disease)

Sxs:

  • Spontaneous Hemarthrosis - bleeding into joint space
  • Bruising and bleeding easily

Dx:

  • ↑ aPTT
  • normal PT and plts
  • Low Factor 8 or 9 on assay

Tx - replace factor 8 or 9

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14
Q

Hodgkin Lymphoma

A

Bimodal - peaks in 20s then 50s ; a/w EBV

Sxs:

  • B constitutional sxs - cyclical fever, wt loss, chills, drenching, nt swts up/down 2 wks period
  • painless enlarged posterior cervical chain (neck)
  • MC supraclavicular LNs (Virchow’s Nodes)

Dx

  • excisional bx - Reed Sternberg Cells (owl eyes)

Tx

  • 5 year prog - excellent
  • Combo chemo
    • Adriamycin
    • Bleomycin
    • Vinblastine
    • Dacarbazine
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15
Q

Hypercoaguable State:

Eti

A

Platelets - too many or overactive

  • TTP
  • HIT
  • HUS
  • HELLP

Vascular injury - plaques, trauma, or burns

Clotting factors - anti clotting factors

  • Protein C
  • Protein S
  • Antithrombin III deficient

Stasis and Surgery

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16
Q

Idiopathic Thrombocytopenic Purpura

Thrombocytopenia

A

Autoimmune rx to platelets usu after viral illness (1-2 wks)

Chronic in adults, self limited in children

A/w HIV, HCV, SLE, CLL

Sxs

  • well appearing, mucosa, skin purpura
  • no hemarthrosis

Dx of exclusion

  • +Direct Coombs Test
  • isolated thrombocytopenia (low plts) - normal CBC and peripheral blood smear
  • normal coag studies

Tx

  • Self limited - no tx
  • IVIG = platelets < 50,000 w. C/I to CS, refractor to CS tx, bleeding or will be bleeding
  • Splenectomy - 2nd line tx for pts w/ refractory ITP
17
Q

Iron Deficiency Anemia

A

MC anemia in US, MCC menstrual bleeding, GI bleeding

Sxs:

  • fatigue/weakness, Splenomegaly
  • glossitis, angular chelitis, pica
  • koilonychia - spoon nails

Dx:

  • Low MCV < 80, low MCH (hypochromic)
  • High TIBC
  • low ferritin (low Fe stores)** < 15ng/mL
    • Females - Hgb < 12/ Hct < 37%
    • Males - <13.5 / < 39%
  • target cells

Tx

  • PO Fe TID 325mg - empty stomach, Vit C for absorption
    • 6 wks to correct
    • 6 mos to restore Fe stores
  • IV Fe infusion - need a skin test first
18
Q

Folate Deficiency

A

Cofactor in DNA synthesis = contributes to neural tube bifida

eti - etoh or malnourished, or pregnant women

Sxs

  • same as B12 but no neuropathy sxs

Dx:

  • low folate
  • incr MCV - looks like B12 but no neuro sxs
  • Hypersegmented PMNs
  • elevated homocysteine; normal MMA levels
  • Howell-Jolly bodies**

Tx - PO Folic acid 1-5mg/d

green veggies

19
Q

Myelodysplastic Syndrome

A

Older pop - previous chemo/XRT

Sxs

  • transition to AML leukemia is common
    *
20
Q

Non-Hodgkin’s Lymphoma

A

Diffuse large B lymphocytes, patients > 50yo

increased IMC risk

Sxs

  • painless peripheral lympadenopathy [more disseminated] (axillary, abd, pelvic, inguinal, femora)
    • common spread to stomach
  • B const sxs not common
  • Non-contiguous extranodal spread - GI and skin most common

Dx

  • Excisional bx
  • Staging with CT

Tx

  • Variable cure rates with chemo
  • R-CHOP
    • Rituximab
    • Cyclophosphamide
    • Hydroxydaunomycin
    • Oncovine
    • Prednisone
21
Q

Polycythemia Vera (primary)

A

Primary - Inherited & malignancy of BM - increased production of RBCs (can affect WBC and plts); M>60yo

Sxs

  • Pruritis after hot baths
  • Swelling, blurred vision, ringing in ear, headache
  • rubor on hands and feet, burning pain

Dx:

  • 4 H’s
    • Hypervolemia - incr RBC, Hct > 30
    • Histaminemia - incr histamine d/t release frm mast cells
    • Hyperviscosity - incr Hct = incr viscosity
    • Hyperuricemia
  • Serum EPO lvls reduced
  • ele Vit B12 levels
  • Positive JAK2** tyrosine kinase mutation
  • BM bx

Tx

  • repeated phlebotomy until Hct < 42%
  • Hydroxyurea, ASA

Secondary Polycythemia - acquired dz

  • 2/2 d/t low O2 - (OSA, COPD, CO levels (toll booth)
    • compensatory release of EPO from kidneys - abs of splenomegaly
    • tx - with O2 treatment
22
Q

Protein C and S Deficiency

Hypercoaguable State

A

Protein C - cleaves/inhibits factor V and VIII (coag factors)*

Protein S - inhibits Factor Va and VIIIa, cofactor of Protein C –> reduces thrombin generation

Vitamin K dependent anticoag liver protein - stimulates fibrinolysis and clot lysis (inactivates factor V and VIII)

  • recurrent DVT and PE risk

Dx

  • Protein C/S functional assay
  • decreased C and S activity levels

Tx

  • Heparin and PO anticoag for life
  • Protein C deficiency - risk of skin necrosis while on warfarin
23
Q

Sickle Cell Anemia

A

AA, fam hx, B-globin mutation - hemolytic anemia; Autosomal Recessive

Heterozygous - sickle cell anemia, homozygous - sick cell trait

sxs:

  • Priapism, bone pain, stroke, hypoxia, deH2O
  • Acute chest syndrome
  • Jaundice, pigmented gallstones, splenomegaly
  • Delayed physical development

Dx:

  • Hemoglobin electrophoresis - Hemoglobin S
  • Hct 15-30%
  • Sickle RBCs, Howell-Jolly bodies, target cells
  • Regular slit exam for retinopathy

Tx

  • Hydroxyurea
  • vigorous H2O
  • Morphine - RBC tranfusion
  • Cure - stem/bone marrow transplant
24
Q

Hemolytic Anemia - Thalassemia

A

Genetic - microcytic; Lowest MCV; hypochromic

only 2 B globin genes instead of 4 - change stability of Hgb = hemolysis

Beta Thalassemia Mino

  • Most severe, Mediterrean, Italian, greek AA, - lifelong microcytic anemia + folic acid supp FTT
  • HgB A2 and F

Beta Thalassemia Major

  • Cooleys anemia - facial malformation, cog delays, bone dz/osteopenia
    • BM transplant, iron chelation to liv e>30 yo

Alpha Thalassemia

  • Silent > Mild > Major
    • Chinese and SE Asians - blood transfusion, folic acid supp
  • Major - hemoglobin Bart’s (Hydrops fetalis)

Dx - periph smear - Target cells and basophilic stippling

Tx

  • acute - blood transfusion
  • Asymp - PO Fe repletion
  • Poor prognosis - most die In 20-30s from Infx, liver or heart failure
25
Q

Thrombotic Thrombocytopenic Purpura (TTP)

Thrombocytopenia

A

low Platelets + anemia + schistocytes on smear; 20-50 yo

Hemolytic Uremic Syndrome (HUS) if childe w/ Low platelets + anemia + renal failure (diarrhea)

Eti - after drugs - quinidine, cyclosporine, pregnancy, estrogen, plavix

inhibition of ADAMTS13

Sxs:

  • Purpura + FAT RN
    • Fever
    • Anemia
    • Thrombocytopenia
    • Renal failure
    • Neurological symptoms

Dx:

  • CBC - ↓ Platelets
  • Schistocytes (RBC fragments)***
  • neg Coombs test

Tx

  • Emergent plasmapheresis** until plts are back to normal
  • Corticosteroids
26
Q

Vitamin B12 deficiency

A

Cobalamine - autoimmune destruction of gastric parietal cells => atrophic gastritis => no Intrinsic Factor (IF) = no Vit B12 absorption

Pernicious Anemia - autoimmune, a/w gastric cancer & IF antibodies

2/2 causes - gastric bypass sx, celiac dz

Sxs:

  • Weakness
  • gums bleeding
  • sore tongue - Glossitis
  • peripheral neuropathy/ paresthesias*
  • Depression, Dementia
  • Late - AMS, neurological deficients = irreversible

Dx:

  • MCV > 100, low B12, high
  • Hypersegmented neutrophils
  • MMA and homocysteine elevated (not in folate)
  • positive Schillings test*** for pernicious anemia
  • IF antibodies = pernicious anemia

Tx

  • IM B12 1000mg q 5 wks lifelong
  • years to replete stores
27
Q

Von Willebrand’s Disease (vWD)

Clotting Factors Disorder

A

MC genetic bleeding disorder, Autosomal Dom

plts can’t adhere to vessel wall = bleeding doesn’t stop as quickly

Sxs/ Dx

  • Hx of bleeding post cut or incr menstrual bleeding, no hemathrosis
  • petechia, small amt of superficial bleeding
  • ↑ PTT, prolonged bleeding time (extrinsic)
  • low vW Factor
  • low Factor 8

Tx:

  • Desmopressin/DDAVP
  • avoid ASA or NSAIDs
28
Q

Thrombocytosis

A

Sustained platelet production;

Uncommon (50-60), Platelet count >500k

Sxs

  • PE and DVT** = main COD
  • Headache as 1st presenting sx
  • Splenomegaly
  • Burning of palms and soles => ASA relieves

Tx

  • Hydroxyurea - suppresses plt count
29
Q

Multiple Myeloma

A

>65yo AA Men; Plasma cell proliferate

Sxs

  • osteolytic bone lesions
  • bone pain, hyperCa, renal failure
  • Cord compression, bone fractures
  • new ARF + compression fractures **

Dx

  • Bence jones proteins - clonal spike on plasma electrophoresis
  • NO FEVER, incr alk phos, splenomegaly

Tx

  • Stem cell transplant
  • Chemo - thalidomide
30
Q

Sideroblastic Anemia

A

Lead exposure in kids

Sxs

  • abd pain, diffic concentrating
  • low IQ, gait ataxia
  • episodic paralytic ileus***

Dx

  • Basophilic stippling*** on smear
  • serum Fe nl
  • Bone marrow - ringed sideroblasts on prussian blue stain

Tx

  • chelation therapy
  • remove
31
Q

Hereditary Spherocytosis

A

Mild hemolytic anemia - Autosomal Dom

Defect in RBC surface membrane

Sxs

  • abn, oval shaped hgb- dense and lack central pallor
  • Hemolysis in body is in state of stress
  • Malaise, jaundice, high # of gall stones - cholecystectomy

Dx - periph smear osmotic fragility test

high RDW

Tx - splenectomy, folate replacement

32
Q

Hemochromatosis

A

Autosomal recessive = excess Fe absorption => accumulation of Fe in organs => fibrosis d/t hydroxyl free radicals

Sxs

  • Liver - main organ - Cirrhosis & HCC x 200x
  • Pancreas - DM (fe deposits in pancreas)
  • Heart - CMO, CHF, arrhythmias
  • Joints - arthritis - 2nd/3rd metacarpo joints, hips, knees
  • Skin - bronzeline

Dx

  • ↑ Elevated ferritin (may be >1,000 ng/mL)
  • ↑ Elevated fasting serum transferrin
  • ↓ Decreased total iron binding capacity
  • Prussian blue stain - brown spots inside hepatocytes

Tx - Phlebotomy or deferoxamine

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