Treatments for Various Disorders Flashcards
(32 cards)
Hereditary Spherocytosis
Splenectomy- spherocytes will persist and will have Howell-Jolly bodies in RBCs on smear w/ increase Hb conc. (elevated MCHC) but anemia resolves since splenic macrophages are no longer consuming/lysing the spherocytes
G6PD Deficiency
Stop offending agent and treat the underlying cause
Must test enzymes WEEKS after crisis
Warm AIHA
- Determine and tx underlying cause (SLE, idiopathic, CLL, methyldopa, fludaribine)
- Prednisone
- IVIG (high dose)
- Splenectomy
- Folic acid (b/c can become folate depleted with increased RBC production)
- Blood transfusion
Cold Agglutinin Disease
- Keep Pt. warm, treat underlying cause
- Monoclonal IgMk: CLL, NHL, Waldenstroms
- Polyclonal: Mycoplasma, younger, acute onset, severe
Paroxysmal Cold Hemoglobinuria
Supportive treatment, self limited 2-3 weeks (assoc w/syphillis or viral infection)
Iron Deficiency Anemia
Oral or IV Fe (ferrous sulfate)
- Hb increase 1.5 dL/week, 4% Hct increase/week
- Increase reticulocytes after 1 week
Anemia of Chronic Disease
- Address the underlying cause (AI, cancer) to decrease hepcidin levels
- Exogenous EPO helpful in a subset of patients esp. cancer
Sickle Cell
Acute Crisis: IV fluids, O2, pain control, RBC exchange transfusions or simple transfusions
- Chronic transfusions
- Hydroxyurea- increases HbF
- HCST transplant (poor outcomes)
- Fe overload management due to chronic transfusions w/ ferrochelation
Beta Thalassemia Major/alpha Thalassemia (HbH)
Chronic transfusions- tx is the COD
- Hydroxyurea (beta thal)
- Chronic transfusions eventually cause hemochromatosis
DIC
Treat the underlying cause + supportive care i.e. repletion of consumed factors
TTP
- Measure ADAMTS13 + Ab to confirm Dx
- Give FFP b/c it has ADAMTS13
- Plasmapheresis b/c it gets rid of Ab and gives back ADAMTS13
HUS
- Supportive care
- Dialysis if needed
- No Abx as may prolong infection
- Platelets contraindicated
Atypical HUS
- Deficiency of complement factor H (CD46) not caused by E.Coli
- Tx like TTP w/ plasmapheresis
vWF Disease
Desmopressin (DDAVP): ADH analog which increases vWF release from the weibel palade bodies of endothelial cells
Hemophilias
Give recombinant factor that is missing!
A= 8, B=9, C= 11, 13 = factor 13 def
HIT
- Stop all Heparin
- Start an IV direct thrombin inhibitor
Follicular Lymphoma
t(14;19) BCL2–IgH
- Rituximab or low dose chemo
- Tx if symptomatic
Chronic Granulomatous Disease
- Bactrim (Trimethoprim-Sulfamethoxazole) for bacterial prophylaxis
- Voriconazole for fungal prophylaxis
- IFN-gamma to up-regulate granulocyte killing A
Cyclic Neuotropenia
- Give G-CSF at 1-2 day intervals
- This decreases the length of the cycle and increases the amplitude of the waves (increases baseline neutrophil count) which reduces infectious complications
X-Linked Aggamaglobulinemia (Burton Syndrome)
IV/SubQ Ig replacement therapy every 2 weeks-1 mo. since NO Igs are produced due to B-cell maturation dysfunction
SCID
- Sterile Isolation (bubble boy)
- Stem Cell transplant can be curative
- Death within 1 year if not treated
Wisckott Aldrich Syndrome
- Splenectomy and BMT can prolong survival
- Complications of WAS are bleeding and infection from thrombocytopenia + defective humoral and cell mediated immunity
CML
Imatinib
- 1st line tx for CML, high rates of molec remission w/ single agent use
- Dasatinib for CML in which abl binding clef mut. to resist imatinib
Essential Thrombocythemia (ET)
- Manage reversible CVD risk factors ( pt. run risk of bleeding or thrombosis)
- High risk pt. - Low dose aspirin + hydroxyurea (limits platelet production)
- Int. Risk pt.- Low dose aspirin + cytoreduction (if they have some other CVD risk factors)
- Low risk pt.- Low dose aspirin
