IRON Deficiency

Epidemiology #1 Cause of Anemia World Hookworm USA GI/Mences Pregnancy, Children, Vegan Pathophysiology *75% stored in Hgb *25% stored Liver/Kidney/Spleen *Proximal Small Bowel (1mg/day) *RBC Production Clinical **Pica Ice & Clay **Koilonychia Nail Spooning **Plumber Vinson Syndrome Dysphagia, Esophagitis, Web *Glossitis Shiny, Red, Painful *Chelitis Cracks in corners Diagnosis *Microcytic Hypochromic *Serum Ferritin *TIBC *Bone Marrow Aspiration* Gold Standard Treatment *Red Meat *325mg PO TID x 6 months

G6PD Deficiency Favism

#1 Enzyme Def in WORLD *Autosomal Recessive X-linked *MALES* *Mediterranean *Black Males *Fava Beans, Humus *Premature Destruction of RBC due to Oxidative Stress* *Pentose Phosphate Pathway* G6PD Enzyme (Maintain/Promote NADPH) NADPH (Maintain/Produce Glutathione) *UTI. PNA, DM, IBD, BP* Glutathione (Protects RBC from OXIDATION) *Asymptomatic #1* *Mild Transient* **Jaundice/Icterus** **Splenomegaly** *Anti-Malarials, *Vitamin K *Nitrofurantoin *Nalidixic Acid (Quinolones) *Methylene Blue *Chloramphenicol *Sulfonamides/Sulfonylureas *Sulfasalazines *Thiazides *Infection (Salmonella, E.Coli, GABHS) *Broad Beans* *Naphthalene (Moth Balls) *Rapid Fluorescent Spot Test Detects NADPH * Serum G6PD Assay (#1) *Reticulocytes ***Heinz Bodies*** *Bite Cells *Schistocytes *Indirect Billirubin *LDH *Haptoglobin **N CBC btw Crisis** *NOT FATAL* *AVOID Triggers* *Vaccinations*

ACUTE Iron Supplement Folate Supplement

Warm AIHA
*Autoimmune *Acute or Chronic *WOMEN *Middle Age *SLE, Leukemia, Lymphoma *50% Idiopathic ***IgG Antibody*** *>98.6 F *Extravascular Hemolysis* Spleen rips off IgG attached outer layer leading to *SPHEROCYTE* Partial Phagocytosis *Intravascular Hemolysis* IgG + MAC *Rapid Onset *Jaundice/Icteric *Splenomegaly *Petechiae/Purpura ***(10%)Evans Syndrome*** IgG attaches to Platelets Thrombolytic Anemia **Normocytic Hyperchromic* *Reticulocytes *Indirect Billirubin *LDH *Haptoglobin *Schistocytes, *Spherocytes *Nucleated RBCs (SEVERE) Lymphoma, Leukemia **Direct Antiglobulin Test** Direct Coombs Test (+) = Clotting (-) = Micro Coombs Test #1 Prednisone #2 Splenectomy Often Controls Dz #3 Immunosuppresives *IV Immune Globulin* Rituximaub #4 Plasmapharesis **Transfuse (CONSULT)** **Good Prognosis** w/ Comorbidity 1

Cold Agglutination AIHA

Epidemiology *Primary* Idiopathic Monoclonal Ab (CHRONIC) *W>50 *Secondary* 1Infection (#1 EBV, Mycoplasma) 2Neoplasm (ALL,CLL) 3Systemic Autoimmune Monoclonal (Adult CHRONIC) Polyclonal (Children ACUTE) Pathophysiology ***IgM Antibody*** *<98.7 F* *Extravascular Hemolysis* LIVER (Kupffer Cells) C3b Receptor *Intravascular Hemolysis* (Hemoglobinuria) Clinical *Jaundice **Dark Urine** (Hemoglobinuria) **Acrocyanosis** Diagnosis *Direct Coombs Test* **RBC Agglutination at Room Temp** *Reticulocytes *Indirect Billirubin *LDH *Haptoglobin *Schistocytes, *Spherocytes **CXR /**EKG *FALSE Megaloblastic Anemia* Agglutination looks BIG Treatment **Supportive** *Avoid Cold * Steroids *Splenectomy *Plasmapharesis Temporary **Transfuse (CONSULT)** **NO Chemo**

SICKLE CELL
*African #1 *MALARIA Adaptation *Autosomal Recessive CAUSES *Dehydration *Hypoxia *Stress *Acidosis *Heterozygous (AS) Hgb *ETOH Carrier *Cold 2 (AS) = 25% Homozygous *Pregnancy *Infection *Folate *Homozygous (SS) Hgb *50% die < 20yrs *50yrs MAX Lifespan *Gallstones* *RBC 10-20 day lifespan *POLIMERIZATION of RBC *Homozygous Onset < 1yr of life (#1) Vaso-Occlusive Crisis ***PAIN*** *Dactylitis *Ulcers (Medial Malleolous) *Bone Avascular Necrosis *H Shaped Vertebrae *Retinal Hemorrhage *Splenomegaly/Hepatomegaly *Renal Failure *Priapism *Chest Pain *CVA, SAH, SZ, Palsy Hematologic Crisis * **SYNCOPE*** * Acute Anemia Exacerbation Splenic Sequestration Aplastic Bone Marrow Parvo B-19 Virus (Fifths Dz) Slap Cheek Infectious Crisis *Due to Functional Asplenia IgM/Oponization Encapsulated Bugs *H. Flu, Strep PNA, E. Coli, Staph A. H&P *Pain, Fever, Cough, Urinary, Weakness, Neck Stiffness, Blurred Vision, Palpitations *Previous Transfusions *Precipitating Event *Vaccinations *JAUNDICE/ICTEROUS* 1) *Sickle Cell Screening Test* Quick 2) *Hgb Electrophoresis* Gold Standard *WBC, Plt, H&H *Reticulocytes *Indirect Billirubin *LDH *Haptoglobin *Howell-Jolly Bodies *Target Cells (Codocytes) *Schistocytes **PREVENTION #1** *PO/IV Fluids *O2 *NO *Narcotics *Antiemetics *Transfusion *Antibiotics ADMIT * Relief p 2 Doses * Maintain Hydration *NON-Extremity PAIN OUTPATIENT *Folate *PO Analgesics *Antibiotics *Vaccines *Hydroxyurea (Hgb F) CA Risk ***Pt. Education*** *Seek care EARLY 2

Folate Deficiency Anemia

Epidemiology *Need 50-100mcg Qday to replenish loss (Urine & Bile) *Citrus, Greens, Meat *Pregnant, Elderly, ETOH, Infancy, Malignancy, Diet, Absorption, Acid, Sprue, Anticonvulsants, PPI, Zinc, Circulation, Thyroid, Trimethoprine, Methotrexate, Sulfonamides Pathophysiology *PABA *Impaired DNA Synthesis *Neural Tube Defects Spina Bifida Clinical **N/V/D** **Anorexia **Abd Pain **Dark Dorsal Skin *Glossitis *Angular Chelitis *Fever Diagnosis *Ovalocytes *Dacrocytes *Hypersegmented Neutrophils * Serum Folate (Acute)<3ng * RBC Folate (Average)<140ng *N Serum B12 *N MMA *Homocysteine *Bone Marrow Biopsy Treatment *Diet *Supplement (IM/SQ/PO)

B12 Deficiency Anemia

*Need 1mcg Qday *Milk, Eggs, Poultry, Meat **Mylenation** *Vegans, Elderly Brain/Spinal Cord *Celtic, Scandinavian *Atrophy/Loss of Gastric Mucosa *Acid IF (Parietal)B12 *Chronic Pancreatitis *Zollinger Ellis Syndrome *wt (10-15lbs) *Parasthesias *Proprioception *Psychosis *Urinary/Bowel Retention *Vibratory Sense *Glossitis *Waxy lemon yellow skin *Premature White Hair *Ovalocytes *Dacrocytes *Hypersegmented Neutrophils *Serum B12 *N Folate *MMA *Homocysteine *Bone Marrow Biopsy ***Schilling Test*** 1) IM B12 (Saturate) 2) PO B12 & Cobalt 3) 24hr UA **Cobalt in Urine = (+) IF/(-) Test ** Cobalt in Urine = (+) Test *100mcg IM Qday x 1wk *100mcg IM Qwk x 5-6wks *100mcg IM Qmonth x Life OR *PO 25-200mcg Qday *Multivitamins MVI-12 / Cernevit-12 *Folate Supplements *Outpatient Follow-up*

Von Willebrands Dz
Epidemiology Pathophysiology #1 Bleeding Disorder in WORLD *VWF Plt ADHERENCE *Autosomal Dominant *VWF OR ABNORMAL *Young Girls Menarche *Binds/Protects Factor VIII *Compromised SECONDARY Hemostasis INTRINSIC Cascade Type 1 (#1) Quantitative () Type 2A Qualitative (SMALL Multimers) Type 2B Qualitative (SPONTANEOUS Clotting) Cleared by Spleen Type 2N Qualitative ( Bind/Stabilize Factor VIII) Type 2M Qualitative (BIG Multimers) Type 3 Quantitative ( VWF) Clinical *Menorrhagia *Nosebleeds *Gum Bleeds *Bruising **Aspirin **Birth Control *Usually MILD* Diagnosis *RISOCETIN COFACTOR ASSAY* Abx that causes coagulation *1HYPO *2A HYPO *2B HYPER *2N NORMAL *2M HYPO *3 PLT 2B Factor VIII 2N & 3 PTT 2N & 3 PT Normal *VWF *Bleeding Time (PRIMARY) Treatment *Avoid Aspirin, NSAID *DDAVP (ADH)* *Release () VWF & Factor VIII ** 2B** *FFP *Factor VIII Concentrates *Antifibrinolytics (AMICAR) Stops TPA *GOOD Prognosis 1 & 2A

Hemophilia A & B
*Autosomal Recessive *X-Linked *MALES *Inability to create cross linked fibrin (INTRINSIC) Hemophilia A * OR Factor VIII Hemophilia B Christmas Dz * Factor IX *Severe (60%) < 1% Factor *Moderate 1-5% Factor *Mild 6-40% Factor *Neonate Cord Bleeding *Circumcision Bleeding *Gingival Bleeding *SAH (Blood in CSF) *Retroperitoneal Bleed Gray Turners Sign Collins Sign **Spontaneous Hemarthrosis Knee #1 Recurrent Muscle Wasting *Factor VIII/IX *PTT *N Plt *VWF *Factor VII/IX Concentrate *FFP *DDAVP Hemophilia A *Amicar *Vaccinations DEEP SQ