| A | B |
|---|
| Sickle cell Anemia description:normocytic | Genetic moon shaped RBCs , abnormal HGB may have trait ( can still harm/stress episode) mainly African American heritage (1 in 12); can't Dx until > 6 mths old. |
| S/S of Sickle Cell Anemia | Chronic anemia pain (very painful, tx c meds); Cardiovascular: cool extremities, hypoxemia, decreased BP, increased HR; Skin: Pale, Jaundice; Organs: Brain, eyes, bones, liver, spleen |
| Why is sickle cell crisis so painful? | The cells clump together occluding the vein, leading to tissue hypoxia and perhaps death. |
| Diagnostics for Sickle Cell Anemia | HX and physical; Fetal blood cell testing (heredity); peripheral smear; CBC, HGB, electrophoresis (ID the presence of abnorm, Hgb); MRI: DX stroke secondary to blocked cerebral vessels; Doppler studies |
| Medical management of Sickle Cell Anemia | Prevent crisis, pain relief, O2 therapy (avoid hypoxia), Labs daily (CBC), Hydration (IV), early recognition, immunize, quick Tx of infections, skin care ABCs |
| Macrocytic (megaloblastic) anemia description | Overly large (unusual shaped RBCs) normal in numbers, normal Hgb, B12 and folate deficiency causes: Deficiency of intrinsic factor, lack of intake, heredity. Total gasterectomy (vit B12, intrinsic factor) men & women >50 y/o |
| S/S of Macrocytic anemia | general s/s if anemia from tissue hypoxia. GI: sore tongue (beefy, red tongue), anorexia. Neuro: Parathesias, muscle weakness. |
| Pernicious Anemia description | Shilling test, morphology. Medical MGMT: Diet high dose oral or IM (Cobalamin for life) Iron; folic acid; Transfusion, mouth care,(sores), Diet |
| What does a shilling test observe? | amount of vitamin B12 over 24 hour period |
| Polycythemia description: | Malignant RBCs, Hyper-viscosity- 2 types, incresed RBC, WBC and platelet counts |
| Polycythemia vs. Pancytopenia | Polycythemia: type of anemia (malignant RBC); Pancytopenia: low number of RBC, WBC, Platelets |
| Diagnostics of Polycythemia | Incresed Hgb, RBC and EPO; Increased WBC and Platelets; Bone marrow biopsy. |
| H & P of Polycythemia | older male, splenomegaly, headache, vertigo, pruritis (increased histamine levels), painful burning of hands and feet. |
| Medical management of Polycythemia | Phlebotomies: decrease Hgt; Meds (immune suppressants) benedryl; hydration; anticoagulation; stem cell transplant. |
| Bleeding disorder Hemophilia | Inherited (males); deficiency in clotting factors hem a and b; spontaneous and uncontrolled bleeding. |
| Hemophilia A- clotting factor missing? | Factor VIII |
| Hemophilia B( Christmas)- clotting factor missing? | Factor IX |
| Diagnostics and tests/labs for hemophilia: | H & P (genetic testing); Chorionic villus sampling (amniocentesis); decreased factor 8 or 9; prolonged PTT (bleeding time); PT; Platelet counts normal; liver biopsy |
| Management of Hemophilia | early DX/ RX prevent bleeds ( no IM injection, no rectal temps, paper tape, no NSAIDS, no contact sports) clotting factors (replace); pain RX prevent complications (immune/ nutrition); Meds: desmopressin (DDAVP) gets factor 8 out of the liver, Cryoperecipitate (factor 8), fresh frozen plasma (FFP); gene therapy |
| First seen in children - WHY? | Circumcision; cardinal sign: hematoma look to joint when kid falls or bumps it ( called hemarthroses) |
| Bleeding disorder Von Willebrand description: | genetic (most inherited bleeding disorder) missing Von Willebrand factor |
| Diagnostics for Von Willebrand: | H & P for easy bruising, epitaxis adolescent girls, prolonged bleeding time (PTT) |
| Bleeding disorder DIC description: | Acute and life threatening; caused by trauma, clotting system accelerated so decreases the fibrin production and lessens the clot integrity. |
| Diagnostics of DIC | H & P bleeding with no tx of same acute illness; oozing and bleeding from everywhere. decreased platelets, prolonged pt and ptt |
| Medical management for DIC | support and FIND UNDERLYING PROBLEM! |
| Bleeding disorder Thrombocytopenia description | Increased destruction of platelets (most common bleeding disorder in children) 60-80% will completely recover. |
| Diagnostics for Thrombocytopenia: | H & P follows viral infection, ecchymoses, petechiae, nose bleeds; Lab: decreased platelets, normal WBC, Hgb,; bone marrow biopsy. |
| Medical management of Thrombocytopenia | steroids, immunoglobulins, spleenectomy |
| Stem Cell transplant aka bone marrow transplant: | may be necessary for tx of immunodeficiency disease; Aplastic anemia and leukemia; Sources: bone marrow, blood, cord blood |
| Transplant may be Autologous or allogeneic: | Autologous: pt marrow taken, TX, stored and later re-infused after chemo. Allogenic: matched donor, Compatible human leukocyte antigen (HLA) |
| Transplant procedure: | 1. chemo and/or radiation kill circulating blood cells and diseased bone marrow. (takes 4-12 days, strict isolation dt no immune system, marked pancytopenia) 2. transfused with donor cells. 3. If successful: healthy bone marrow makes new healthy cells, (takes 2 weeks to mature, BRM given to stimulate production(Neupogen and Epoienten)) Embryonic stem cells: harvested |
| Major risks post transplant: | Leukopenia: decrease total WBC count; Neutropenia: decrease neutrophils (low # shows risk for infection) minor infections can cause sepsis!! |
| White blood cell dysfunction leukemia description: | Genetic mutations, immune system and chromosomal abnormalities. Acute and chronic ALL and AML |
| ALL (acute lymphocytic): AML (Acute myelogenous) | ALL: Most common cancer in kids very high survival rate, immature, lymphocytes in bone marrow, abrupt onset, fever, bleeding, bone/joint pain. AML: most common cancer in adults, abrupt onset bleeding, infections uncontrolled proliferation of myeolobasts precursors to granulocytes. |
| Diagnostics for ALL/AML | Morphology blood work: CBC/Differential decreased RBC, platelets, hct, Hgb Electrolytes and liver studies. Immunological studies: subtypes of cells?markers, nuclear medicine scans; staging (1-4) |
| Medical Management of ALL/AML | Cytotoxic chemo (multiple drugs) Stages: 1- Introduction: Dangerous medications. 2-Consolidation: starts after chemo, additional courses of therapy. 3- Maintenance: lower doses of same drugs q 3-4 weeks. Targeted therapies: monoclonal antibodies. |
| Chemo meds for ALL/AML | Daunorubican (Cerabidine) Doxorubican (Adriamycin) Methotorxate Prednisone |
| Hodgkins Lymphoma description: | Malignancy of lymphoid system- Reed- Sternberg cells in lymphnodes; enlarged nodes common ages: 15-35 and >50; Epstein- Barr virus; genetic predisposition, environmental factors. S/S- weight loss, fever, night sweats, cervical region enlarged, inguinal lymph nodes enlarged, may have fertility issues. (TX) may need chemo/ radiation, biopsy needed (bone marrow, nodes) CT/MRI staging (1-4); good prognosis if caught early. |
| Non-Hodgkin's Lymphoma description: | Malignancy of lymphoid tissue, not Hodgkins d/t diffuse of large b-cell blast cells common in those who have immune disorders ( immunosuppressant therapy) various types, slow progression, less responsive to TX |
