Heme-Onc Flashcards
carboxyhemoglobinemia
causes - smoke inhalation (smokers, though this is not enough to cause poisoning, additional CO will tip these individuals over), defective heating systems, motors in poorly ventilated areas
px
- mild - headache, confusion, malaise, dizziness, nausea
- severe - seizure, syncope, coma, myocardial ischemia, arrhythmias
- (secondary) polycythemia in chronic poisoning
dx - ABG (pulse ox does not distinguish between O2 and CO), EKG, cardiac enzymes if indicated
tx - 100% high-flow oxygen, intubation/hyperbaric oxygen therapy for severe cases
polycythemia vera
myeloproliferative disorder, JAK2 mutation
pts are asx - but have signs of increased blood viscosity
- transient neuro sxs, thrombosis, aquagenic pruritus, gout, erythromelalgia (burning cyanosis in hands/feet), HTN
- facial plethora, splenomegaly
- transient vision loss - due to microvascular sludging and not thromboembolism
- increases in all 3 cell lines are common
tx - phlebotomy, hydroxurea (if increased risk of thrombus)
prostate cancer
limited prostate cancer - flutamide and LHRH agonist prolong survival
PSA recurrence after radical prostatectomy - salvage radiation
- salvage therapy is treatment for disease when std treatment fails
pt with painful bony mets after andogen ablation (orchiectomy) –> radiation therapy
- etidronate disodium (bisphosphonate) - reduces bone resoprtion, helps with bony mets, slower onset of action than radiation therapy
leukemia
AML - most common adult leukemia, age 65 yo
- px - fatigue, pancytopenia (anemic, easy bleeding/bruising, infection)
- DIC can occur in APML - prolonged PT/aPTT, hypofibrinogenemia
- APML - RAR mutation - treat with all-trans retinoic acid
- labs - leukocytes can be high/low/nl, elevated LDH, myeloblasts with Auer rods
- dx - bone marrow bx (will show hypercellularity, myeloid blasts)
- blasts accumulated in bone marrow and peripheral blood
CML - BCR-ABL 9-22
- mature/maturing granulocytes - absolute basophilia, early neutrophil precursors
- px - splenomegaly
(abdominal fullness), anemia, thrombocytosis, leukocytosis
- WBC > 100 K - BCR-ABL encodes an active tyrosine kinase –> leukemogenesis
- tx - imatinib - not curative but can induce long-term remission
CLL - most common type of leukemia in US, median age 70
- px - LAD, hepatosplenomegaly, fatigue, often asx
- dx - severe lymphocytosis (mature cells) and smudge cells, flow cytometry
- prognostic - median survival is 10 yrs
- worse prognosis if multiple chain LAD, hepatosplenomegaly, anemia and TCP
- complications - infection, autoimmune hemolytic anemia, secondary malignancies (Richter transformation)
Hairy cell leukemia
- splenomegaly, pancytopenia
Lymphoblastic leukemia - blasts on peripheral blood smera, pancytopenia and associated sxs
Von Willenbrand disease
associated with factor 8 deficiency, plts are nl or mildly decreased
- aPTT abnormal
lymphoma
Hodgkins - mediastinal mass, pancytopenia, elevated LDH, B symptoms, painless LAD
- will NOT have massive lymphocytosis
- curable lymphoma in young pts - 90% 5-yr survival
- pts treated before 30 are at risk for secondary malignancies due to chemorads
Non-Hodgkins
- LAD, splenomegaly, B symtoms
multiple myeloma
bone pain
macrocytic anemia
folate, B12, pernicious anemia
- elevated homocysteine in both, elevated methylmalonic acid in B12 def
- pernicious anemia - most common cause of B12 def in white northern Europeans, will also have other autoimmune diseases (thyroid, vitiligo)
myelodysplastic syndromes - elderly, pancytopenia and macrocytosis, dysplastic cells in bone marrow
AML
drug-induced - hydroxyurea, zidovudine, chemo
liver disease, alcohol abuse
hypothyroidism
anemia
Low MCV - iron deficiency, lead intoxication, thalassemia, sideroblastic anemia
- ferritin reflects total body Fe stores
- Fe deficiency anemia - low Fe and ferritin, low transferrin sat, high TIBC
- thalassemia - microcytic target cells (even more microcytic than Fe deficiency), high Fe and ferritin, high transferrin, low TBC
- screening - CBC in non-AA pts, CBC and Hgb electrophoresis in AA pts
- anemia of chronic disease - low Fe, nl-high ferritin (acute phase reactant), …
Normal MCV
- Low retics* - leukemia, aplastic anemia, infection, med side effect, CKD
- leukemia and viral infections - suppression EPOsis
- CKD - normochromic normocytic hypoproliferative anemia, give EPO, goal is Hgb of 10-11.5
- sometimes these pts can get concurrent Fe deficiency (Fe rapidly depleted during hematopoiesis and in chronically ill pts) - give IV Fe to CKD pts with Fe deficiency
- High retics* - hemorrhage, hemolysis
- intrinsic hemolysis - inherited defects of Hgb, membrane, or enzymes
- hereditary spherocytosis - AD, pts of n euro descent, splenomegaly
- Short life-span of spherocytes and chronic hemolysis –> jaundice, dark urine, pigment gallstones (acute chole)
- Labs - elevated MCHC, increased osmotic fragility, abnormal eosin-5-maleimide binding test
- Tx - folate supplementation, splenectomy
- extrinsic - autoimmune, paroxysmal nocturnal hemoglobinuria (defect in CD55, and subsequent complement-mediated destruction)
- autoimmune hemolytic anemia - splenomegaly due to RBC entrapment
- warm - drugs (penicilin), viral infections, SLE, immunodeficiency, CLL. IgG, tx with corticosteroids, splenectomy for refractory disease. Complications are VTE and lymphoproliferative disorders.
- cold - infections (Mycoplasma PNA, mono), lymphoproliferative diseases
- px - livedo reticularis with acral cyanosis with cold exposure, IgM, tx is avoidance of cold, can ive rituximab
- complications - ischemia, peripheral gangrene, lymphoproliferative disorders
High MCV - B12, folate
- pts on certain anti-epileptics can have impaired absorption of folic acid in SI
heparin
heparin-induced TCP
- type 1 - direct heaprin effect on plts, presents within first 2 days, plt count normalizes with continued heparin therapy
- type 2 (more serious) - antibodies to platelet factor 4-heparin complex –> plt aggregation, TCP, and arterial and venous thrombosis
- px - 5-10d after initiation of therapy
enoxaparin = LMWH
why warfarin and heparin co-therapy - warfarin initially knocks out protein C –> risks of thrombosis and skin necrosis
G6PD deficiency
XR, AA men
enzyme involved in creating NADPH - cofactor required to create glutathione and prevent oxidation of Hgb
- Hgb denatures into Heinz bodies - disrupts RBC membranes, hemolysis
- triggered by oxidant drugs (antimalarials, sulfas), infection, fava beans
- px - anemia, dark urine, jaundice, abdominal/back pain
- G6PD assay can be normal during attack - erythrocytes that are G6PD deficiney are hemolyzed during attack and circulating retics have normal G6PD levels
tx - stop offending agent, supportive care
categorizing therapies
induction therapy - initial dose to rapidly kill tumor cells
adjuvant - treatment in addition to standard therapy (occurs at the same time)
consolidation therapy - multidrug therapy
neoadjuvant therapy - treatment before standard therapy (ex preop radiation)
TTP = thrombotic thrombocytopenic purpura
decreased AdamsTS13 level - uncleaved VWF multimers –> plt trapping and activation –> small vessel thrombi –> shearing of RBCs and end organ damage
- acquired autoantibody or hereditary
- triggered by infections (HIV), malignancy, medications
px - adults
- pentad - hemolytic anemia (high LDH, low haptoglobin) with schistocytes, TCP (increased bleeding time, normal PT/PTT), renal failure, neuro manifestation, fever
- get peripheral blood smear
tx - plasma exchange (to replenish AdamsTS13 and removal of autoantibodies), glucocorticoids, rituximab
- dont give plts unless pt has significant bleeding
- without treatment - mortality is as high as 90%
v. s. HUS - HUS will not have neuro findings or fever, associated with Ecoli O157:H7 - also primarily affects children
hemophilia A and B
XR
A = decreased/absent factor 8
B = decreased/absent factor 9
px - delayed/prolonged bleeding after mild trauma/procedure
- hemarthrosis, hemophlic arthropathy, IM hematomas, GI/GU bleeding
- labs - long aPTT
tx - replace missing factor
VW disease
AD, abnormalities in plt plug formation –> prolonged mucosal bleeding –> menorrhagia
