Hilgerson 1/2

Question 1

Acute Myelogenous leukemia (AML)***

Answer 1

• 80% of acute leukemias in adults
• over age of 60

Question 2

Etiologys of AML

Answer 2

• Ionizing radiation (atomic bomb survivors, prior radiation therapy)
• benzene
• increased incidence with Down’s patients
• myeloproliferative or myelodysplastic syndromes
• chemotherapy can cause tx related AML

Question 3

typical clinical presentation of AML***

Answer 3

• Malaise, pallor, weight loss, fever and BLEEDING (not hyperviscosity/thrombotic problems)
  
  • bleeding via nosebleeds, melana, hematuria, purpura

Question 4

those with treated AML die of..***

Answer 4

INFECTION

hemorrhage from thombocytopenia

Question 5

What is a morphologic indication of AML**

Answer 5

• AUER RODS ON PERIPHERAL SMEAR

Question 6

Acute lymphoblastic leukemia epidemiology***

Answer 6

• 20% of adults leukemias
• primarily a disease of kids (75% of patients < 15)

Question 7

Etiology of ALL

Answer 7

• ionizing radiation
• increase incidence with down’s patients

Question 8

clinical presentaiton of ALL**

Answer 8

• Similar to AML
  
  • malaise, pallor, weight loss, fever and BLEEDING!!!
• CNS meningeal involvement (more often in ALL) may produce stiff neck, papilledema and cranial nerve involvement

Question 9

What phsyical findings are more common in ALL than in AML**

Answer 9

• lymphadenopathy occurs in > 50% of ALL
  
  • uncommon in AML
• Hepatosplenomegaly > 2/3rds of ALL
  
  • hepatomegally uncomoin in AML

Question 10

prognosis of ALL**

Answer 10

better in children

much WORSE IN INFANTS AND ADULTS

Question 11

Myeloproliferative syndromes tend to have what..

Answer 11

BASOPHILIA tends to occur in ALL of the myeloproliferative syndromes

Question 12

Chronic Myelogenous leukemia (CML) serology/cytogenetics

Answer 12

• leukocytosis (sometimes 50 to 600K
• Increased basophils, eosinphils and nucleated RBC
• low leukocyte alkaline phosphatase
• > 30% blasts in peripheral blood or marrow is the strict defintiion of a BLAST CRISIS

CYTOGENETICS

• PHILADELPHIA CHROMOSOME –> CML!!! (t(9,22) or bcr-abl oncogene)

Question 13

CML presentation (actie phase)

Answer 13

• lethargy, weight loss, increasing abdomen girth, sweating, easy brusing/bleeding HEPATO-SPLENOGEALLY
• Diagnose = t(9,22); Ph(+) xome; LOW LEUK ALK PHOS

Question 14

What are some compliactions of CML

Answer 14

• almost inevitably become BLASTIC and converts to ACUTE LEUKEMIA (80% AML, 20% are ALL)

Question 15

Polycythemia vera general

Answer 15

• Hb and Hct ABOVE NORMAL
  
  • relative polycthemia = caused by dehydration from GI loss or insensible fluid loss

Question 16

primary and secondary erythropoietin

Answer 16

• Primary
  
  • Erythropoietin (AKA EPO) = independent Red blood cell production (low EPO = PCV)
• Secondary
  
  • exogenous EPO admin
  • excessive EPO production
    
    • tissue hypoxia (high altitude, pulm dz, sleep apnea)
    • Autonomous secretion from selected tumors

Question 17

PCV clinical presentation

Answer 17

• patietn is typically older
• INTENSE PRURITIS AFTER BATHING
• patients appear plethoric; RUDDY complexion; SPLENOMEGALY
• Serology: classically elevated Hgb/Hct, elevated RBC mass

Question 18

TX of PCV

Answer 18

• Periodic phblebotomy (goal Hct <45%)

Question 19

Chronic Idiopathic Myelofibrosis

(aka myelofibrosis with myeloid metaplasia , MMM)

Answer 19

• “tear drop” RBCs –> seen in all disease which infiltrate the BM, but especially striking numbers in MMM
  
  • characteristic DRY TAP due to increased collagen and reticulin fibers

Question 20

Essential thrombocythemia

Answer 20

• platelets = 600K to 4 million
• better defintion: myeloproliferative disorder with persistent thrombocytosis

Question 21

complications of thombocythemia

Answer 21

• THROMBOSIS (occur most often)
  
  • cloths
• Hemorrhage (includes epistaxis, GI bleeding)
  
  • dont function properly
• Splenomegaly
  
  • filter for all blood cells

Question 22

essential diagnosis of thrombocythemia

Answer 22

• Plt count > 600K
• megakaryocytic hyperplasia
• splenomegaly
• absence of Ph(+) xome –> rule out ALL
• normal RBC mass –> rule out stuff
• Exclusion of all causes of reactive thrombocytosis
• stainable iron in marrow