Tumor lysis syndrome risk
- high
- intermediate
- low
High:
- AML, ALL
- Lymphomas
- intermediate risk with elevated electrolytes
Intermediate:
- solid tumors
- lower grade lymphomas
Low risk:
- Solid tumors
Treatment of TLS
Rasiburicase- causes breakdown of the urate therefore use if kidney disease and hyperuricemia already. Do no use in G6PD deficiency
Hydration with UOP 80-100cc/hr
Allopurinol: use if no evidence of hyperuricemia. Increases the excretion of urate. Do no use in kidney disease
Myelofibrosis
- Symptoms
- Diagnosis - peripheral smear
- bone marrow biopsy
Myeloproliferative disorder
clonal proliferation of abnormal stem cells produce cytokines that promote fibrosis of the bone marrow resulting in:
- splenomegaly/hepatomegaly (from extra-medullary hematopoiesis)
- — may develop portal hypertension
- anemia (normocytic) - may have initial leukocytosis
- thrombocytopenia - may have initial thrombocytosis
- normal MCV
On peripheral Smear:
- Tear drop cells
- circulating erythroblasts and myeloid precursors
- giant platelets
Bone Marrow:
- Dry tap
Commonly JAK2 mutation
treatment:
- Hydroxyurea and ruxolitinib
- Allogenic HSCT if <60yo
Myelofibrosis Treatment
Hydroxyurea
Ruxolitinib (JAK 2 inhibitor)
HSCT <60yo
** PV at risk for developing “post pv myelofibrosis”
Myelodysplastic Syndromes
- Symptoms
- Diagnosis
- bone marrow biopsy
- Flow cytometry
Due to Ineffective hematopoiesis
symptoms:
- associated with cytopenias
Diagnosis
- at least 2 lines of cytopenias
- Anemia with Elevated MCV (normal b12, folate, no alcoholism)
- NO hepatosplenomegaly
Bone Marrow:
- Hypercellular (intra-medullary apoptosis; therefore cytopenias)
- Flow cytometry: look for 5q - specific for MDS
Myelodysplastic Syndrome Treatment
IPSS score to determine risk and subsequently treatment:
- low risk: no treatment
- If <60yo and High risk: HSCT
- if >60yo and High risk: 5-azacytidine
if 5q- lenalidomide
Graft Versus Host Disease- Acute
Graft T cells attack the Gut, Skin, Liver, Renal system- (all over)
Acute: Within 100 days of transplant Skin: bullous rash, TENS Liver: Elevated LFTs Gut: Dyspepsia, N/V Renal: SLE like Nephritis
Treatment:
- High dose steroids
- Cyclosporine and MTX
Graft Versus Host Disease- Chronic
Can happen at any time.
* fibrotic changes more than inflammatory as in acute
Skin: Hypo/hyperpigmentation, lichen planus, fibrotic changes Liver: Elevated LFTs Gut: Esophageal webs and strictures Lung: BOOP Joints: Stiffness, fascitis
Treatment:
Localized: topical steroids
Diffuse: Steroids, cyclosporine
Essential Thrombocythemia
- symptoms
- Diagnosis
Myeloproliferative disorder
Symptoms:
- Headaches, dizziness, erythromelalgia, blood clots (arterial and venous.
- IDA anemia that corrects with Iron, but platelets are still increased
- Splenomegaly
- At risk for acquired vWD due to consumption
Diagnosis:
- elevated platelets >600k on 2 separate occasions, 1 month apart.
- JAK 2 mutation may be present
Treatment:
- Low risk: ASA
- High Risk: HU
- plateletpharesis if stroke, MI, TIA, GI bleeding
Hairy Cell Leukemia
B cell accumulate in the bone marrow- cytopenias and splenomegaly.
Sx:
- associated with cytopenias
- Abd pain due to splenomegaly
Diagnosis
- Peripheral smear: hairy Cells
- Bone marrow biopsy with Flow cytometry
Treatment:
- Chemo
Leukoreduced
When the RBCs separated from whole blood, WBCs can remain with the RBCs.
- Leukoreduction further removes WBCs but not ALL the components of WBCs
- CMV resides in WBCs therefore reduces the risk of transmission in CMV negative patients.
Indicated for
- frequent transfusions
- CMV negative at risk (AIDs, transplant)
- potential transplant candidates
- Previous febrile non-hemolytic reactions
Washed
When RBCs separated from whole blood, plasma can remain. Washing removes the plasma (proteins)
Indicated for
- IgA deficiency
- Complement related autoimmune hemolytic anemia
- Continued allergic reactions to transfusion despite pre-treatment
Irradiated
When RBCs separated WBC components remain
- Irradiation removes all WBC components
Indicated for
- BMT patients
- Acquired or genetic Cellular immunodeficiency
- blood donated by 1st or 2nd degree relatives
Polycythemia Vera
Myeloproliferative disorder
Jak2 mutation
Suspect with Hemoglobin 18.5 (m), 16.5(w) and after secondary causes have been eliminated
sx: plethora, erythromelgia, pruritis, splenomegaly, thrombosis or bleeding. May have stroke, DVT, or budd chiari syndrome.
tx: therapeutic phlebotomy to lower to hemocrit <45.
Low dose ASA
