OMED Hematology Oncology Flashcards
Folate deficiency Path: Pt: Dx: Tx:
Path: leafy green, 3-6 week stores, pregnancy
Pt: tea+toast diet, EtOh, pregnancy
Dx: Folic acid levels decreased, but normal methyl malonic acid
Tx: folate, 1 mg po
B12 deficiency Path: Pt: Dx: Tx: Fu:
Path: 3-10 yr storage, animal products, neuro symptoms
Pt: vegan, pernicious anemia, crohn’s, gastric bypass
Dx: B12 levels decreased, increased methyl malonic acid
Tx: B12, PO= nutritional, IM= impaired absorption
Fu: never choose schilling test on exam, prolonged deficiency causes DCMLS
Pernicious Anemia Path: Pt: Dx: Tx: fu:
Path: Antibodies against parietal cells, no intrinsic factor, no B12 absorption
Pt: weakness, sore tongue, paresthesias
Dx: Ab-intrinsic factor, Ab-anti parietal
Tx: IM B12, cannot treat pernicious anemia
F/u: gastric cancer
non megaloblastic anemia Path: Pt: Dx: Tx:
Path: liver diz, EtOh, medications: (AZT, HAART, 5 FY, ARA-C), metabolic syndromes (Lesch Nyhan, Hereditary orotic aciduira)
Iron deficiency anemia Path: Pt: Dx: Tx:
Path: slow bleed, consumption of iron stores
Pt: women- menorrhagia, older men = colon cancer
Dx: iron studies- ↓iron, ↑TIBH, ↓ Ferritin
Tx: iron 2-6 wks for anemia, iron 2- months for iron stores
Anemia of chronic disease Path: Pt: Dx: Tx:
Path: chronic inflammation
Pt: anemic
Dx: iron studies: iron studies- ↓iron, ↓TIBH ↑ Ferritin. bone marrow biopsy, rarely needed.
Tx: treat underlying condition, EPO for severe cases
Thalassemia Path: Pt: Dx: Tx:
Path: genetic mutations, loss of genes, 4 alpha, 2 beta Pt: -Asx: 1 alpha deletion (asymptomatic) -Minor: 2 alpha deletions, 1 beta deletions -Major: 3 alpha deletions, 2 beta deletions -Dead: 4 alpha deletions Dx: - Fe normal - Ferritin normal - TIBC normal - Hgb Electrophoresis Tx: -minor: do nothing -major = transfusions = deferoxamine
sideroblastic anemia path: Pt: Dx: Tx:
Path:
-irreversible: B6, cancer
-reversible: lead, EtOh, copper, isoniazid
Pt: anemia
Dx:
-Fe ↑, Ferritin normal, TIBC normal
-BEST: bone marrow biopsy showing ringed sideroblasts
Tx: remove exposure, give back B6, try to treat cancer
Sickle Cell
Path:
Path:
- Autosomal recessive
- HgbSS
- Valine –> glutamine
- Sickling under stress (acidosis, dehydration)
Sickle Cell
Pt:
Pt: Emergency acute- *acute chest = MI, CHF *acute brain = CVA *priapism
Hospitalized Acute-
*vaso-occlusive crisis
Chronic-
- asplenia
- avascular necrosis
- osteomyelitis
Sickle Cell
Dx:
Tx:
F/u
Dx:
*1st- smear = sickled cells –> used on first crisis, not subsequently. if they were diagnosed previously, dont use smear.
*
Best: Hgb electrophoresis (Sc, SS)
-use 1st time only
Tx: hydroxyurea = ↑HgbF, ↓HgbSS, IVF, pain control, exchange transfusion in emergency
f/u: iron overload (deferoxamine)
G6PD deficiency Path: Pt: Dx: Tx:
Path: ↓ G6PD, hypoxemic = hemolysis
Pt: African American Males, dapsone, TMP-SMX, nitrofurantoin
Dx: 1st smear = bite cells, heinz bodies.
best: G6PD levels 6-8 weeks
Tx: supportive, avoid stressors
Hereditary spherocytosis Path: Pt: Dx: Tx:
Path: deficiency in spectrin, ankyrin, pallidin
Pt: hemolysis, spherocytosis
Dx:
smear–>spherocytes –> osmotic fragility test (+)
Tx: folate + Fe, splenectomy
Paroxysmal Nocturnal Hemoglobinuria Path: Pt: Dx: Tx:
Path: PIGA deficiency Pt: Paroxysmal (once in awhile) Nocturnal (only at night) Hemoglobinuria (dark urin)
Dx: flow cytometry, ↓CD55, ↓CD59
Tx: Biologics (Eculizumab)
Warm Autoimmune Hemolytic Anemia Path: Pt: Dx: Tx:
Path: IgG (Cancer,Drugs,Rheumatoid) Pt: Hemolysis Everywhere Dx: Coombs Test IgG Tx: -First line: steroids -Recurrent: splenectomy -Severe: IVIg -Refractory to splenectomy: rituximab
Cold autoimmune hemolytic anemia Path: Pt: Dx: Tx:
Path: IgM (Mycoplasma, mono) Pt: Hemolysis in the cold -tips of digits and nose Dx: First line: Coombs test negative for IgG Tx: -Avoid the cold -Refractory: rituximab
Microangiopathic Hemolytic anemia Path: Pt: Dx: Tx:
Path: Schistocytes = MAHA Pt: TTP versus DIC Dx: -Decreased hemoglobin (both) -Decreased PLT (both) -Decreased fibrinogen (DIC) -Increased INR (DIC) -Increased split products (DIC) Tx: See thrombocytopenia
Acute myelogenous leukemia Path: Pt: Dx: Tx:
Path: acute = Blasts Myelogenous= Neutrophils Leukemia= Cancer in the blood Pt: -Acute, age 67 -Exposure: Benzene radiation -CML: Blast crisis Dx: -First: Smear= blasts -BM Bx > 20% Tx: M3: Vit A ) auer Rods on bx (-) M3: chemo
Acute lymphocytic leukemia Path: Pt: Dx: Tx:
Path: -acute = blasts -lymphocytic = leukocytes -leukemia = cancer in the blood Pt: acute, age 7 Dx: -First: smear = blasts -BM Bx > 20% blasts + cALLa and TdT Tx: -chemo -PPx CNS ARA-C +/- radiation
Chronic myelogenous leukemia
Path:
- Chronic =mature cells
- Myelogenous =neutrophils
- Leukemia =cancer in the blood
pt: Chronic, age 47
Dx
- Dff (way more cells then should be)
- BM Bx = Philadelphia + t(9, 22) + BCR – ABL
tx: Imatinib (tyrosine kinase)
f/u: blast crisis
Chronic lymphocytic leukemia
Path:
Pt:
Dx:
Tx:
path:
- Chronic equals mature cells
- Lymphocytic equals lymphocytes
- Leukemia equals cancer in the blood
pt: Chronic, Age 87
dx: diff (Way more cells that should be)
tx:
- 65+ and asx = nothing
- 65+ and Sx = chemo
- < 65 + donor = HSCT
Contiguous lymph node spread, B sxs, painful lymphadenopathy with EtOh
Pt:
Dx:
Tx:
Hodgkin’s lymphoma
Pt: nontender LAD
Dx:
-excisional biopsy showing Reed Sternberg Cells
-Staging with PET/CT or CT Chest/Abdo/Pelvis
Tx:
IIa or better = radiation
IIb or worse = chemo (ABVD, BEACOPP)
Hematogenous spread
usually no B sxs
starry sky on biopsy
Extranodal diz
Pt:
Dx:
Tx:
NHL
Pt: nontender LAD +/- sxs
Dx:
- excisional biopsy
- NO reed sternberg cells
- stage PT/CT or CT chest/abdo/pelvis
Tx:
- IIa or better = chemo
- IIb or worse, chemo = ABVD or BEACOPP
Staging of lymphoma I-IV
LNs location relative to diaphragm
I = I = not applicable II = 2+ and same side diaphragm III = 2+ and opposite side of diaphragm IV = Mets = N/A
Adverse Effects
cisplatin- bleomycin- adriamycin/doxorubicin- vincristine/vinblastine- cisplatin- cyclophosphamide-
cisplatin- ototoxicity
bleomycin- pulmonary fibrosis
adriamycin/doxorubicin- cardiac toxicity
vincristine/vinblastine- peripheral neuropathy
cisplatin- nephrotoxicity
cyclophosphamide- hemorrhagic cystitis
70 yo with hypercalcemia, renal failure, anemia, and bone pain
Path: Pt: Dx: Tx: f/u
Multiple myeloma
Path: IgG, monoclonal expansion of plasma cells
- Ig = recurrent infections
- bence jones = AKI
- osteoclasts = lytic lesions
Pt: 70s+ male with CRAB symptoms (hypercalcemia, renal failure, anemia, and bone pain)
Dx:
Spep+
Upep+
Skeletal survey +
BM Bx > 10 % plasma cells
Tx: above 70 - chemo, no donor. below 70, donor: HSCT
F/u: melphalan + steroids, thalidomide, bortezomib
Incidental IgG spike without CRAB symptoms
Path:
Pt:
Dx:
Tx:
MGUS, early myeloma
Path: increased IgG
Pt: 85+ yo, no CRAB symptoms
Dx: Spep+, Upep- , skeletal survey - , BM Bx < 10% plasma cells
Tx: watch and wait, converts to MM 2%/yr
Constitutional symptoms, hyperviscosity syndrome, IgM
Pt:
Dx:
Tx:
f/u
Waldenstrom’s
Path: IgM
Pt: Constitutional symptoms, hyperviscosity syndrome
Dx: Spep + , Upep - , skeletal survey - , BM Bx > 10% lymphoma
Tx:
-Rituximab - chemo
-Hyperviscosity = plasmapheresis
F/u: blast crisis
Thrombophilic Disorders listed
Factor V Leiden (mutation) Prothrombin 20210A (mutation) Protein C def (level) Protein S def (level) Antithrombin def (level)
Who gets warfarin in a DVT?
All patients except metz cancer pts
“provoked 3 months”
“unprovoked 6 months”
recurrence lifetime
DVT prophylaxis
risk
cancer
risk:
- trauma
- surgery (10x)
- LWMH PPx
- LMWH –> warfarin
Cancer
LMWH ppx
LMWH treatment (no warfarin in met cancer)
fever, anemia, thrombocytopenia, renal fialure, neuro symptoms
Path:
Pt:
Dx:
Tx:
TTP
Path: hyaline clots, adamts-13 deficiency
Pt ("FAT RN")
Fever
Anemia
Thrombocytopenia
Renal failure
Neurological symptoms
Dx:
- CBC: ↓ platelets
- Smear: schistocytes
- PT/PTT: normal
- fibrinogen: normal
- D-dimer: normal
Tx:
-exchange transfusion
F/u: NEVER platelets
SICK AS SHIT, SEPSIS, ICU SHOCK, THEN THEY START BLEEDING
Path:
Pt:
Dx:
Tx:
DIC
Path: fibrin clots, tremendous catastrophic injury
Pt: SICK AS SHIT, SEPSIS, ICU SHOCK, THEN THEY START BLEEDING
Dx:
- CBC: ↓ platelets
- Smear: schistocytes
- PT/PTT: ↑
- fibrinogen: ↓
- D-dimer: ↑
Tx:
- Fix underling disease
- give blood products
thrombocytopenia following heparin exposure
Path:
Pt:
Dx:
Tx:
HIT
Path: antibodies to platelets
Pt: heparin –> (7-14 days)–> Plts ↓ by ~50%–> new clots
Dx: antiplatelet factor 4 Elisa +, confirm with serotonin release assay
Tx: stop heparin, argatroban –> warfarin INR 2-3
Woman with autoimmune disease and ↓ platelets
Path:
Pt:
Dx:
Tx:
Path: antibodies to platelets, splenic destruction
Pt: Woman with autoimmune disease and ↓ platelets
Dx: Diagnosis of exclusion
Tx: steroids, IVIg, splenectomy, rituximab
woman with heavy menstrual periods and easy bleeding
Path:
Pt:
Dx:
Tx:
Path:
- MC bleeding disorder
- Deficiency of vWD
- autosomal dom
Pt: platelet bleed, normal count, can have factor VIII def
Dx:
- 1st - CBC = normal count
- best = vWD assay
- must have ↑ PTT from factor VIII deficiency
Tx: DDAVP (Desmopressin)
Bleeding AND clotting, hemarthrosis (bleeding), arterial thrombosis (clotting)
Path:
Pt:
Dx:
Tx:
Antiphospholipid antibody syndrome
Path: LUPUS
Pt: Bleeding AND clotting, hemarthrosis (bleeding), arterial thrombosis (clotting)
Dx: Pt/PTT/INR, mixing studies that do not correct, Vpier Venom Assay
Tx: heparin to warfarin bridge for APLA, treat lupus
Acquired Factor Deficiency Path: Pt: Dx: Tx:
Path: liver dysfunction, warfarin, Vit K def
Pt: long ICU stay, on warfarin, has cirrhosis
Dx: PT/PTT/INR
Tx: Vitamin K
Bleeding Disorders: list them
VWD ITP TTP DIC Glanzmann's Bernard-Soulier Hemophilia Liver Disease Vit K Deficiency
Thrombophilia (over-clotting) diseases: list them
Factor V Leiden Protein C Deficiency Protein S Deficiency Antithrombin Antiphospholipid Syndrome
gingival, vaginal, skin bleeding are signs of a problem in which ° of hemostasis?
primary hemostasis = platelet bleeding
normal platelet count = platelet function error
low platelet count = platelet destruction/consumption (thrombocytopenia)
primary hemostasis with a normal
normal platelet count =
primary hemostasis with a low platelet count =
primary hemostasis = platelet bleeding
normal platelet count = platelet function error
low platelet count = platelet destruction/consumption (thrombocytopenia)
main causes of thrombocytopenia
A. destruction (ITP, TTP, DIC, HIT)
B. production (aplastic, mylodysplastic syndromes, cancer)
C. sequestration -
splenic dysfunction or liver disease
Bernard Soulie vs Glanzmann’s
BS has one b: problem is Glycoprotein I B
so
GT Glycoprotein II B 3A
PT/PTT abnormalities are signs of a problem in which ° of hemostasis?
secondary hemostasis (factors) THEN PERFORM
-mixing study
↓
bleeding continues- indicates presence of inhibitor
BUT IF
↓
-mixing study: BLEEDING TIME CORRECTED--> Hemophilia A/B,
Vitamin K def, Warfarin therapy
DIC, WBD (Factor VIII instability)
Causes of a primary hemostasis dysfunction with a normal platelet count =
NSAIDs, ASA, clopidigrel
uremia, VWD
Causes of a primary hemostasis dysfunction with a low platelet count =
Destruction of platelets (ITP, TTP, HTP, DIC)
Underproduction of Platelets (myelodysplastic syndromes, cancer)
Sequestration of platelets
(splenic dysfunction and liver disease)
How to diagnose a bleeding disorder
CBC
PT/PTT (then mixing study if abnormal)
Platelet count- normal or low?
Hemoglobin-
normal or precipitous drop?
