Treatment - Endo & Heme Flashcards
hyperthyroid
- MC therapy: RADIOACTIVE IODINE + HORMONE REPLACE
- METHIMAZOLE OR PROPYTHIOURACIL (PTU)
- BB for symptoms
- Thyroidectomy if radioactive iodine c/i (pregnancy)
*PTU PREFERRED IN PREGNANCY, ESP 1ST TRI
thyroid storm (thyrotoxicosis crisis)
anti-thyroid meds: IV PROPYLTHIOURACIL (PTU) or methimazole
- BB for symptomatic therapy
- IV GLUCOCORTICOSTEROIDS (supportive - inhibits peripheral conversion of t4 to t3 and impairs thyroid hormone production)
- AVOID ASPIRIN (causes increased T3/T4)
hyperprolactinemia
dopamine agonists (dopamine inhibits prolactin)
CABERGOLINE or bromocriptine
(after stop offending agent), surgery for some patients
lead poisoning
remove source of lead; CHELATION TX IF SEVERE
-Treatment is oral succimer or IV EDTA (calcium disodium edetate, given after dimercaprol)
alpha thalassemia
-mild (a-trait) - no tx needed
-moderate: folate (if retic count high), avoid Fe supplement
-severe: blood transfusions weekly, vit c, folate supplement
Iron-chelating agents (prevents Fe overload), +/-splenectomy
-allogeneic BM transplant is the definitive tx of major
beta thalassemia
MAJOR
- periodic blood transfusions, Vit C, folate supplementation, avoid excess Fe intake
- Iron-chelating agents: IV Deferoxamine, PO Deferasirox
- Splenectomy if refractory
- Allogenic BM transplant definitive
sickle cell
-IV HYDRATION AND O2 FIRST STEP IN PAIN CRISIS (reverses/prevents sickling)
-NARCOTICS for pain, avoid meperidine
+/-RBC TRANSFUSION IN SEVERE
- HYDROXYUREA - reduces freq of pain crisis (inc RBC water, dec RBC sickling, inc HgbF - resistant to sickling)
- FOLIC ACID - needed for RBC production and DNA synth
- CHILDREN IMMUNIZE FOR S.PNEUMOCOCCUS, Hib, N.MENINGOCOCCUS
- CHILDREN: PROPHYLACTIC PCN UNTIL 6 YO
hereditary spherocytosis
- FOLIC ACID (not curative but helpful - maintains RBC production and DNA synthesis)
- SPLENECTOMY TX OF CHOICE IN SEV DISEASE
autoimmune hemolytic anemia
- WARM: CORTICO 1ST LINE –> splenectomy or Rituximab
- COLD: AVOID COLD EXPOSURE –> +/- Rituximab
paroxysmal nocturnal hemoglobinuria
- ECULIZUMAB (anti-complement C5 ab)
- Prednisone dec hemolysis; BM transplant
TTP
-PLASMAPHERESIS TX OF CHOICE
-IMMUNOSUPPRESION - CORTICOSTEROIDS
-no platelet transfusions (may cause thrombi formation)
+/- splenectomy if refractory
hemolytic uremic syndrome
- OBSERVATION IN MOST (IV fluids for renal perfusion)
- PLASMAPHERESIS +FFP if severe, or complications
- ABX MAY WORSEN CONDITION (INC VEROTOXIN RELEASE BC OF CELL LYSIS)
DIC
- treat underlying cause!
- FFP IF SEVERE BLEEDING +/- platelet transfusion
- thrombosis: +/- heparin in severe cases
ITP (idiopathic/autoimmune thrombocytopenic purpura
- tx- support/observe if >20K platelets and no bleed
- children: OBSERVE –> IVIG
- adults: CORTICOSTEROIDS –> IVIG –> splenectomy if refractory
*transfusion of platelets on if <20K to prevent spontaneous intracranial hemorrhage (only if bleeding after cortico + ivig)
hemophilia A
- FACTOR 8 INFUSION - to levels 25-100% prn
- DESMOPRESSIN (DDAVP) - transiently increases Factor 8 and vWF release so may be used prior to procedures
hemophilia B
- FACTOR 9 INFUSION
- DESMOPRESSIN NOT USEFUL!
von Willebrand deficiency
- avoid aspirin, assess bleeding times before procedures
- mild: no tx needed
- mod: DESMOPRESSIN (DDAVP) inc vWF, Factor 8
- sev: absent vWF –> supplement w/ vWF-containing products and Factor 8 (DDAVP NOT HELPFUL)
polycythemia vera
- PHLEBOTOMY MANAGEMENT OF CHOICE (until hct <45), low dose ASA
- myelosuppresion: HYDROXYUREA
- allopurinol if pt hyperuricemic
- Ruxolitinib is a JAK inhibitor
secondary erythrocytosis
tx- underlying disorder, smoking cessation
hereditary hemochromatosis
- PHLEBOTOMY WEEKLY until depletion of iron –> maintenance 3-4x year for life
- Chelating agents if unable to do phlebotomy (anemia)
- Tx complications, test relatives, genetic counsel
- NO ETOH or VIT C
coagulopathy of advanced liver dz
- FRESH FROZEN PLASMA w/ active bleeding or invasive procedure (to replace coag factors); vit K won’t correct
- Cryoprecipitate if low fibrinogen
factor 5 leiden
- high risk –> indefinite anticoag (may need thromboprophylaxis during pregnancy to prevent miscarriages)
- mod risk –> prophylaxis during high-risk procedures
protein C deficiency
-Heparin –> PO ANTICOAG FOR LIFE
may devo WARFARIN-INDUCED SKIN NECROSIS
DVT
Treatment is anticoagulation
Warfarin is contraindicated in pregnancy
methemoglobinemia
Methylene blue
fanconi anemia
Treatment is bone marrow transplant
antiphospholipid antibody syndrome
anti-coagulation
