Heme Exam 2 Disorders PDF

Disorder Risk Factors S&S Diagnostics Rx Ddx Myeloproliferative Disorders Polycythemia JAK2 genetic mutation Sx: H/H: ↑ (hgb> 16, Hct > 48)...

Disorder Risk Factors S&S Diagnostics Rx Ddx Myeloproliferative Disorders Polycythemia JAK2 genetic mutation Sx: H/H: ↑ (hgb> 16, Hct > 48) Low risk: Spurious Vera (genetic predispo & h/a, dizziness WBC: ↑ - Phlebotomy (1 unit per week polycythemia acquired mutation→ Tinnitus Platelets: ↑(normal morphology) until Hct < 45) (caused by Myeloproliferative born with it and it occurs Blurred vision RBC morphology: normal - low dose aspirin (1 mg) dehydration) neoplasm that after exposure) Fatigue - stop smoking develops 2/2 Epistaxis (nose bleed) EPO: ↓ Secondary: acquired *predominantly males* Pruritus (Especially after B12: ↑ High risk: caused by low O2 abnormality in shower) Uric acid: possible ↑ - phlebotomy levels in the body, hematopoietic stem Low risk: 60 & hx of Conjunctival injection - stop smoking thrombocytosis x all 3 stem cell lines thrombosis Engorged retinal veins (RBC most Palpable spleen Maybe: antihistamines for pruritis prominent) *need 3 major or 2 major + 1 & allopurinol if high uric acid minor to diagnose* Primary → NO ↑ EPO *15 year survival rte* Essential 50 - 60 year olds Thrombosis Platelets: ↑ Hydroxyurea (or anagrelide) Secondary causes Thrombocytosis WBC: ↑ maybe Aspirin 81 mg BID for sx of high platelets women> men Erythromelalgia (red hands) RBC: normal (inflammation, Myeloproliferative Bleeding (mucosal) → rare IDA, UC, disorder of JAK2 mutation Splenomegaly common JAK2 mutation 15 yr prognosis, can transform splenectomy) megakaryocytes in into myelofibrosis, leukemia, BM → high splenic infarction PV (RBC is main platelets diff)  Disorder Risk Factors S&S Diagnostics Rx Ddx Myelofibrosis (weird morphology) WBC Disorders Leukocytosis Neutrophilia: (ANC< What does the pt look like? WBC < 20,000 → may be 7700) retested in a week Elevated WBC - Infection (bacterial, - weight loss, fever, LAD count (>11,000) HSC, varicella, TB, - rash, drug use, infection WBC > 100,000 → emergent mono) evaluation Most commonly - inflammation neutrophilia, - medications ALC > 50,000 → emergency sometimes (catecholamines, evaluation (higher = less likely lymphocytosis glucocorticoids, lithium) to be reactive) - asplenia ↑ BM production - cigarette smoking CBC (reactive or - stress/ exercise CMP autonomous) - malignancies BM biopsy (demargination of Flow cytometry WBC → unbound Lymphocytosis: (ALC> Molecular & genetic testing to epithelium) 4000) - viral illnesses (EBV, *BLASTS = BAD* CMV, HIV) - pertussis, toxoplas., cat scratch - medical stress, asplenia, thymoma - malignancy Leukopenia Neutropenia: (ANC Fever Is it isolated or is pancytopenia Neutropenia: 110 IM/ SQ (daily x 1 wk, weekly x ↓ production of intrinsic Neuro: paresthesias, gait B12: ↓ 1mo, then monthly) (differentiate w In animal products factor disturbances, cognition Homocysteine: ↑ neuro signs and disturbances (b12 breaks this down) correct/ treat underlying cause b12 levels, B12 absorbed in (GI disorders or Glossitis Methylmalonic acid: ↑ homocysteine & distal ileum resection that interferes Retic: ↓ methylmalonic (requires intrinsic w distal ileal Signs: Peripheral smear: acid) factor from gastric absorption) Slow in onset & proportional macro-ovalocytes & parietal cells) → to degree of anemia hypersegmented neutrophils absorption in liver → DNA synthesis LDH, indirect bili: ↑ WBCs, platelets: low if severe BM biopsy: hyperplasia of RBCs  Disorder Risk Factors S&S Diagnostics Rx Ddx Folate deficiency Inadequate intake Similar to above Hgb: ↓ Oral folic acid, 1 mg daily B12 def MCV: > 110 (diff with neuro Leafy, greens, ↑ demand (preg) NO NEURO SIGNS Folate level: ↓ Prevent during preg, signs, MMA or citrus Peripheral smear: hemodialysis, and pt on meds that folate levels) ↑ loss (hemodialysis) macro-ovalocytes & interfere w absorption Taken in through hypersegmented neutrophils GI → stored in Meds that interfere w GI liver & other body absorption (sulfasalazine, Test for co-existing b12 def tissues → DNA phenytoin, TMP-SMX) synthesis Macrocytic → non- megaloblastic Aplastic Anemia Autoimmune Sx: Pancytopenia: Referral to heme Congenital Anemia: fatigue RBCs & WBCs & platelets: ↓ Bone marrow Viral Neutropenia: infections Erythroid growth factors, myeloid growth factors failure → Pregnancy Thrombocytopenia: easy Retic: ↓ pancytopenia 2/2 Medications, toxins bruising, bleeding Transfuse RBCs & platelets injury or MCV: mildly ↑ Abx if infection suppression of Signs: Immature cells NOT seen on hematopoietic stem Mucosal pallor smear If less than 40 y/o: cells Petechiae (hands & feet) BM transplant (HLA matched) Purpura BM aspiration & biopsy for confirmation → hypocellularity - 80% survival rate 40 or 50% following IgG Ab form 5-14 days post Heparin activation in the absence of heparin exposure Alternative anticoagulant w  Disorder Risk Factors S&S Diagnostics Rx Ddx against hemostasis) direct thrombin inhibitor heparin-platelet (usually unfractionated PF4 Ab (ELISA, confirm with factor 4(PF4) hep) Possible asympto assay) Consult heme complexes *bleeding is NOT common* Use 4T score to predict risk ALWAYS HOSPITALIZE