Treatment - Endo & Heme

Question 1

hyperthyroid

Answer 1

• 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

Question 2

thyroid storm (thyrotoxicosis crisis)

Answer 2

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)

Question 3

hyperprolactinemia

Answer 3

dopamine agonists (dopamine inhibits prolactin)
CABERGOLINE or bromocriptine
(after stop offending agent), surgery for some patients

Question 4

lead poisoning

Answer 4

remove source of lead; CHELATION TX IF SEVERE

-Treatment is oral succimer or IV EDTA (calcium disodium edetate, given after dimercaprol)

Question 5

alpha thalassemia

Answer 5

-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

Question 6

beta thalassemia

Answer 6

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

Question 7

sickle cell

Answer 7

-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

Question 8

hereditary spherocytosis

Answer 8

• FOLIC ACID (not curative but helpful - maintains RBC production and DNA synthesis)
• SPLENECTOMY TX OF CHOICE IN SEV DISEASE

Question 9

autoimmune hemolytic anemia

Answer 9

• WARM: CORTICO 1ST LINE –> splenectomy or Rituximab

- COLD: AVOID COLD EXPOSURE –> +/- Rituximab

Question 10

paroxysmal nocturnal hemoglobinuria

Answer 10

• ECULIZUMAB (anti-complement C5 ab)

- Prednisone dec hemolysis; BM transplant

Question 11

TTP

Answer 11

-PLASMAPHERESIS TX OF CHOICE
-IMMUNOSUPPRESION - CORTICOSTEROIDS
-no platelet transfusions (may cause thrombi formation)
+/- splenectomy if refractory

Question 12

hemolytic uremic syndrome

Answer 12

• 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)

Question 13

DIC

Answer 13

• treat underlying cause!
• FFP IF SEVERE BLEEDING +/- platelet transfusion
• thrombosis: +/- heparin in severe cases

Question 14

ITP (idiopathic/autoimmune thrombocytopenic purpura

Answer 14

• 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)

Question 15

hemophilia A

Answer 15

• FACTOR 8 INFUSION - to levels 25-100% prn

- DESMOPRESSIN (DDAVP) - transiently increases Factor 8 and vWF release so may be used prior to procedures

Question 16

hemophilia B

Answer 16

• FACTOR 9 INFUSION

- DESMOPRESSIN NOT USEFUL!

Question 17

von Willebrand deficiency

Answer 17

• 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)

Question 18

polycythemia vera

Answer 18

• PHLEBOTOMY MANAGEMENT OF CHOICE (until hct <45), low dose ASA
• myelosuppresion: HYDROXYUREA
• allopurinol if pt hyperuricemic
• Ruxolitinib is a JAK inhibitor

Question 19

secondary erythrocytosis

Answer 19

tx- underlying disorder, smoking cessation

Question 20

hereditary hemochromatosis

Answer 20

• 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

Question 21

coagulopathy of advanced liver dz

Answer 21

• FRESH FROZEN PLASMA w/ active bleeding or invasive procedure (to replace coag factors); vit K won’t correct
• Cryoprecipitate if low fibrinogen

Question 22

factor 5 leiden

Answer 22

• high risk –> indefinite anticoag (may need thromboprophylaxis during pregnancy to prevent miscarriages)
• mod risk –> prophylaxis during high-risk procedures

Question 23

protein C deficiency

Answer 23

-Heparin –> PO ANTICOAG FOR LIFE

may devo WARFARIN-INDUCED SKIN NECROSIS

Question 24

DVT

Answer 24

Treatment is anticoagulation

Warfarin is contraindicated in pregnancy

Question 25

methemoglobinemia

Answer 25

Methylene blue

Question 26

fanconi anemia

Answer 26

Treatment is bone marrow transplant

Question 27

antiphospholipid antibody syndrome

Answer 27

anti-coagulation