TREATMENT

PLAN
 Treatment: Cobalamin and Folate
Deficiency
• Treat only with the appropriate vitamin

• A. Cobalamin deficiency:
• -lifelong cobalamin injections
• -Parenteral therapy for initial treatment(severe anemia)
• -malabsorption of Cobalamin or rises serum MMA levels: must be given regular
maintenance Cobalamin therapy (1000 ug Hydroxocobalamin IM once every 3
months)
• -allergic reactions: requires desensitization/antihistamine/glucocorticoid cover.
• -oral therapy: monitor compliance
• -sublingual therapy: for injection difficulty due to bleeding tendency
Treatment: • B. Folate Deficiency
• -oral doses of 5-15 mg folic acid daily
Cobalamin • -continued up to 4 months (all folate-
deficient red cells will have been eliminated
and Folate and replaced by new folate-replete
populations)
Deficiency • -for large doses: cobalamin deficiency must
be excluded first
• -long term therapy: required for uncorrected
underlying cause of deficiency, measure
cobalamin level at regular intervals (yearly) to
exclude the coincidental development of
cobalamin deficiency.
 Treatment: Cobalamin and Folate Deficiency

• -in patients who are severely ill, treat with both vitamins in large doses once
blood samples have been taken for cobalamin and folate assays and a bone
marrow biopsy has been performed.

• -Blood transfusion: unnecessary and inadvisable. If essential, give packed red

cells slowly, 1-2 units only,
• -potassium supplements -to prevent hypokalemia but is unnecessary
• -anti-platelet therapy: for excessive rise of platelets at 1-2 weeks of therapy
Treatment: • Acute painful crisis: vigorous hydration,
thorough evaluation of cause and aggressive
Sickle cell analgesia administration (morphine 0.1-0.15

syndromes
mg/kg every 3-4 hours)
• Bone pain: ketorolac (30-60 mg initial dose,
then 15-30 mg every 6-8 hours)
• Nitrous oxide inhalation: short-term pain
relief (caution: hypoxia and respiratory
depression)
• Emergency rooms: reserved for severe
circumstances (infection)
• Blood transfusion: for extreme cases
Treatment: • Hydroxyurea:
• - mainstay therapy for severe symptoms
Sickle cell • -considered in repeated episodes of acute
chest syndrome (>3 crises/year requiring
syndromes hospitalization

• Bone marrow transplantation

• -provides definitive cure but known to be
effective and safe in children
Treatment: • Medical emergency!
-immediate BT of red cells (paradoxically incompatible
hemolytic blood)

anemia If anemia is not immediately life threatening:

Withhold blood transfusion (compatibility problems may
increase with each unit of blood transfused)

Immediately start prednisone (1 mg/kg/day) and

Rituximab (100 mg/wk x 4) –first line standard
- Prompt remission and reduce relapses

Relapse and refractory to medical treatment:

Consider splenectomy to remove major site of hemolysis

Autologous/allogeneic hematopoietic stem cell

transplantation- for very severe refractory cases
Treatment: • -severe acquired aplastic anemia can be cured
by replacement of the hematopoietic cells by
Aplastic stem cell transplant or suppression of

anemia
immune system

• Hematopoietic Stem Cell Transplantation:

• -best therapy for younger patient with a fully
histocompatible sibling donor

• Immunosuppression
• -standard regimen of antithymocyte globulin
in combination with cyclosporine which
induces hematologic recovery
Treatment: • Red cell transfusion: for symptomatic elderly
patients with severe iron-deficiency anemia and
iron cardiovascular instability.

deficiency • Oral Iron therapy:

anemia •
•
-adequate for asymptomatic patients
-200 mg of elemental iron per day (3-4 iron tablets)
• -goal: to repair anemia and provide stores of
atleast0.5-1 g of iron through a sustained
treatment for 6-12 months.

• Intravenous iron therapy:

• -given to patients who cannot tolerate oral iron
and having a persistent GI blood loss
Treatment: • TKI therapy:
• -imantinib, nilotinib, and dasatinib (frontline therapies)
• Aim: achievement and maintenance of a complete
Chronic cytogenic response

Myeloid • Allogeneic Stem cell transplant

• -curative modality in CML

leukemia • Patient’s on blastic phase:

• -Combinations of chemotherapy and TKIs are used to
induce remission, followed by SCT.

• Chemotherapeutic agents:
• -hydroxyurea and busulfan

• Splenectomy
• -considered to alleviate symptoms of massive
splenomegaly or hypersplenism