Heme Flashcards by Giana Indelicato

Signs and symptoms include: Vary based on severity of factor deficiency 1. Easy bruising at injection sites 2. Prolonged bleeding following circumcision 3. Excessive bruising after child begins walking 4. Mucosal bleeding 5. Prolonged bleeding in any part of the body 6. May have pain at the site of the bleed 7. Hemarthrosis (bleeding into the joint spaces)

Hemophilia

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Particularly effective in areas where fibrinolysis appears to contribute to bleeding., i.e. in mucous membranes, trauma, and dental procedures

Antifibrinolytic Agents

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Diagnostic platelet count <10,000

Chronic ITP

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Moderate; gross bleeding following moderate trauma; some hemarthrosis; rare spontaneous hemorrhage

1-5% Factor VIII/IX

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Management/treatment includes: 1. Children should be screen between the ages of 9-12 months and 1-5 years for at risk 2. Maintain breastfeeding for at least 6 months with iron-fortified cereal by 4-5 months; No cows milk before 1 year, limit to 18-24 oz 3. Administer iron until HMG normal and then additional 3-4 months to replace iron stores; give on empty stomach

Iron-Deficiency Anemia

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Physical findings include: 1. Petechiae, purpura, and ecchymoses 2. Hemorrhages in mucous membranes 3. Pallor usually not present (usually there has been significant bleeding) 4. Splenomegaly

ITP

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Diagnostic tests/findings include: 1. Variation in findings by specific variant as well as within same patient over time 2. Bleeding time - usually prolonged 3. VWF- usually decreased or absent 4. VWF antigen - usually decreased or absent 5. Blood group - VWF decreased in type O

VWD

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Children should with this illness should also receive PPV every 5-10 years as recommended by current guidelines

SCD

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Most severe ocular change in SCD; treated with laser therapy.

Proliferative Sickle Retinopathy

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Prevention includes: Education regarding precipitating factors like dehydration, hypoxia, fever, exposure to extreme temperatures

Acute Painful Events

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Regular infusions of factor VIII or IX given to prevent joint hemorrhage and bleeding episodes; initiated before 3 years old

Primary prophylaxis

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Thrombocytopenia Platelet count associated with spontaneous bleeding; Risk for ICH

<10

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Management includes: 1. PRBCs transfusion 2. Exchange transfusion for severe vaso-occlusive associated problems

Acute Exacerbations of Anemia

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Affinity to platelet binding

Type 2M VWD

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Asymptomatic with sickle hemoglobin 35-45% and hemoglobin A 55-65%; AS

Sickle Cell Trait

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Diagnostic tests/findings include: 1. G6PD fluorescence-based screen (may give false negative) 2. RBC indices during or just after hemolytic episode (Heinz bodies present, fragmented cells and blister cells) 3. Reticulocytosis 4. Normal HMG between episodes 5. Acute self-limiting hemolytic anemia with hemoglobinuria

G6PD

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Referral to hematologist 1. When thrombocytopenia worsens during viral illness or prior to elective surgery, prednisone or IVIG can be administered 2. Low-dose steroids may be necessary for for a prolonged period of time; antacids, H2 blockers, or PPI need to be taken with steroids 3. Rituximab may be effective in refractory instances 4. Splenectomy - treatment of choice when disease is severe or symptomatic (prophylactic PCN/AMX daily)

Chronic ITP

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Severe anemia in-utero caused by alpha thalassemia; fatal without intervention

Hydrops Fetalis

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Curative treatment for SCD that eliminates sickle cell erythrocytes and cellular progenitors and replaces with donor hematopoietic stems cells that give rise to erythrocytes

Stem Cell Transplant

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Initiated for musculoskeletal bleeds

Physical therapy

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Alternative treatment for VWD if desmopressin is not indicated or effective

Factor Concentrate with VWF

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Diagnostic tests/findings include: 1. Anemia, hypochromia, microcytosis, low MCV, target cells, increased reticulocyte

Beta Thalassemia Major (Cooley’s Anemia) and Intermedia

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Symptoms include chest pain, shortness of breath, chills, productive cough, hemoptysis, hypoxia, tachypnea, and wheezing; 1/3 do not exhibit symptoms.

Acute Chest Syndrome

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Physical findings include: Signs of bleeding, including ecchymosis, swelling and pain in joints, and prolonged bleeding from lacerations or injections

Hemophilia

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Hemoglobinopathy caused by decreased synthesis of beta-like globins, ineffective erythropoiesis, and shortened red cell life span

Beta Thalassemia

Initiated by 2 months of age and continued until at least 5 years of age for children with SCD

Prophylactic PCN

Mild to moderate clinical severity with sickle hemoglobin 45-55% and hemoglobin C 45-55%; near normal lifespan; SC

Sickle Hemoglobin C

Signs and symptoms include: 1. Bruising 2. Nosebleeds 3. Bleeding gums and lips 4. Petechiae 5. Menorrhagia 6. Child appears well except for signs of bleeding

ITP

A microcytic, hypochromic anemia caused by inadequate supply of iron, associated with low reticulocyte count and elevated red cell distribution width

Iron-Deficiency Anemia

Acute onset of fever with accompanying pulmonary symptoms including tachypnea, wheeze or cough, and new alveolar infiltrate suggestive of consolidation on chest x-ray; may result from bacterial or viral infection, fat embolism from bone infarction and necrosis, and vaso-occlusion of he pulmonary vasculature.

Acute Chest Syndrome

Management/treatment includes: 1. Generally mild symptoms require minimal intervention 2. Identification and avoidance of foods and drugs that cause hemolysis 3. Transfusion for severe hemolysis 4. Genetic counseling; routine screening not generally recommended

G6PD

Usually marked clinical severity with sickle hemoglobin equal to 80-90%; life span usually about 40 years; SS

Sickle Cell Anemia

Detects elevated blood velocity in middle cerebral arteries secondary to elevated CBF and/or stenosis; completed yearly (toddler-teens) as primary stroke prevention

Trans-Cranial Doppler Ultrasound

Bleeding disorder caused by congenital deficiency or absence of clotting factor VIII or IX

Hemophilia

Hemoglobinopathy that is usually asymptomatic with normal physical examination

Beta Thalassemia Minor (trait), Alpha Thalassemia (trait), HMG C/E

A collection of autosomal recessive, recessive genetic disorders characterized by the hemoglobin S variant of the beta-globin gene

Sickle Cell Disease

Rare form of VWD

Type 2B VWD

Physical findings include: 1. Hyperbilirubinemia in infants - usually associated with Med. and Canton individuals 2. Older children are asymptomatic between episodes of hemolysis

G6PD

Immune-mediated disorder characterized by production of antiplatelet antibodies caused by an autoimmune disorder sometimes related to sensitization by viral infection, most prevalent during early-mid childhood

Immune Thrombocytopenia

Diagnostic tests/findings include: 1. mild or no anemia, increased HbA2, microcytosis, hypochromia No management needed

Beta Thalassemia Trait

ITP that continues between 3-12 months from diagnosis

Persistent ITP

Hemoglobinopathy associated with occasional splenomegaly and microcytic anemia

HMG C/E and A-Thalassemia Hb H

Isolated thrombocytopenia (platelet count of 100,000 in the absence of other causes)

Primary ITP

Severe; spontaneous hemarthrosis and deep-tissue hemorrhages

\< 1% Factor VIII/IX

Mucocutaneous bleeding: 1. Epistaxis 2. Easy bruising 3. Petechiae 4. Menorrhagia 5. Gingival bleeding

Thrombocytopenia

Symptoms develop 24-48 hours after ingestion of substance having oxidant properties: 1. Weakness 2. Pale appearance 3. Severe case - blood in urine, yellow discoloration of skin, whites of eyes, and gyms or inside of mouth

G6PD

Sudden intrasplenic pooling of blood leading to acute drop in circulatory volume with rigid distended abdomen. Management includes prompt correction of hypovolemia, RBC transfusion, chronic transfusion therapy for recurrent episodes, and/or splenectomy (\>2 moderate episodes or after 1 severe episode). Must avoid contact sports.

Splenic Sequestration

Diagnostic tests/findings include: 1. Prenatal diagnosis - can be done by fetal blood sampling through fetoscopy or by analysis of DNA obtained through chorionic villus sampling or amniocentesis 2. Diagnostic test - direct assay of plasma factor activity for A or B 3. Screening tests - Activated partial thromboplastin time - prolonged; PT - normal; bleeding time - normal

Hemophila

Factor IX deficiency, Christmas disease; X-linked disorder, with 20-30% spontaneous mutation

Hemophilia B

ITP lasting for more than 12 months

Chronic ITP

Management/Treatment includes: 1. Chronic transfusion protocol - to maintain hemoglobin, support growth, and prevent extramedullary hematopoiesis 2. Chelation therapy to remove excessive intracellular iron 3. Prophylactic PCN 4. Splenectomy may help (all immunizations given and daily PCN/AMX)

Beta Thalassemia Major

Physical findings include: 1. Splenomegaly, occasional hepatosplenomegaly 2. Jaundice, usually mild 3. Abnormal facies secondary to extramedullary hematopoiesis - prominence of malar eminences, frontal bossing, depression of bridge of nose, exposure of upper incisors 4. Growth retardation 5. Bronze skin

Beta Thalassemia Major/Intermedia

Associated with SCD: 1. Pain - most often in bones, but can occur in any part of body; in children \<2 years usually in hands/feet 2. Swelling - sometimes seen at site of pain 3. Low-grade fever-sometimes occurs

Vaso-Occlusive Crisis

An abnormal VWF molecule that does not bind with factor VIII

Type 2N VWD

Signs and symptoms vary with severity: 1. Mild - usually asymptomatic 2. Fatigue 3. Irritability 4. Delayed motor development 5. Decreased attentiveness, shorter attention span, poor school performance 6. Eating nonnutrient substances such as ice, plaster, clay, paint, fabric

Iron-Deficiency Anemia

Signs and symptoms include: Manifestations begin around 4-6 months of life 1. Pale skin or mucous membranes 2. Weakness 3. Characteristic facies (frontal bossing, exposure of frontal teeth, prominence of malar eminences)

Beta Thalassemia Major/Intermedia

An inherited hemorrhagic disorder characterized by defective primary hemostasis and due to a quantitative or qualitative abnormality in blood factor; most common congenital bleeding disorder

Von Willebrand Disease

Mild; severe hemorrhage only following moderate to severe trauma or surgery

6-25% Factor VIII/IX

Administered intravenously or intranasally for mild FVIII deficiency

Desmopressin

Treatment usually not indicated if platelet count \>50,000; considered if platelet count \<20,000, especially with extensive cutaneous bleeding or if protective environment cannot be ensured 1. High-dose corticosteroids for 1-3 weeks with slow wean 2. IVIG 1-3 days; treatment of choice for acute bleeds; platelets may be given but are short-lived 3. Anti-D IG

Acute ITP

Decreased platelet-dependent VWF

Type 2A VWD

Administered to children with frequent SCD complications to decrease hemolysis and painful events; increases production of Hb F, which retards gelation and sickling. May cause neutropenia, thrombocytopenia, anemia, GI disturbance, rash/nail changes, renal failure, splenic sequestration, and headache

Hydroxyurea

All forms of immune-mediated thrombocytopenia except primary ITP

Secondary ITP

Management/treatment includes: 1. Factor IX concentrate intravenously

Hemophilia B

Hemoglobin S results from a single base pair substitution of valine for glutamic acid of the beta globin gene resulting in distorting RBCs into classic crescent or sickle shapes when deoxygenated; this leads to hemolysis and intermittent episodes of vascular occlusion that can cause tissue ischemia and acute/chronic organ dysfunction

Sickle Cell Disease

Associated with SCD: 1. Weakness 2. Irritability 3. Unusual sleepiness 4. Paleness 5. Large spleen 6. Fast heart rate 7. Pain in the left side of abdomen (does not always occur)

Splenic Sequestration

Administered for oral mucosal bleeds

Antifibrinolytic therapy

Normal platelet count

150-400

Antibiotic administered to SCD patient with fever

Ceftriaxone

Diagnostic platelet count \<20,000; often 10,000

Acute ITP

Severe bleeding disorder with deficits in primary and secondary hemostasis

Type 3 VWD

Management includes aggressive identification and treatment of infection, transfusion to improve hypoxia, oxygen supplementation, and mechanical ventilation when necessary. Airway hyperreactivity should be managed with bronchodilators.

Acute Chest Syndrome

Factor VIII deficiency, classical hemophilia that is 3rd most common X-linked disorder and 4x more common than factor IX deficiency

Hemophilia A

Treatment for SCD that binds to iron to facilitate elimination (iron overload); monitor serum ferritin levels and renal/hepatic function. Adverse effects - nausea, diarrhea, liver toxicity

Chelation Therapy

Associated with SCD/Parvovirus B19: 1. Pale 2. Malaise 3. Headache 4. Fever 5. Mild upper respiratory infection symptoms

Aplastic Crisis

Thrombocytopenia Platelet count associated with increased risk of bleeding, GI bleeding, and hematuria

\<20

Management includes: 1. Home-based - analgesia, hydration, heat, localized massage 2. ED - parenteral fluids, analgesia 3. Inpatient - Hydration, parenteral analgesics and NSAID

Acute Painful Events

Started after a joint has developed hemarthrosis; used to prevent further bleeding into the joint

Secondary prophylaxis

Used to treat bleeding complications or as preoperative preparation for Type 1 VWD; contraindicated in the treatment of Type 2B

Desmopressin

Laboratory findings include: decreased hemoglobin (6.5-9.5), increased reticulocyte count (5-20), and increased MCV (\>80)

Sickle Cell Anemia/Sickle Beta Zero Thalassemia

Signs and symptoms vary with associated problems: 1. Hemolysis 2. Chronic anemia 3. Jaundice 4. Aplastic crisis 5. Cholelithiasis 6. Delayed growth and sexual maturity 7. Vaso-occlusion 8. Recurrent acute pain 9. Functional asplenia 10. Splenic sequestration 11. Acute chest syndrome 12. Stroke 13. Hyposthenuria and enuresis 14. Papillary necrosis of kidneys 15. Chronic nephropathy 16. Priapism 17. Avascular necrosis of humeral heads, femoral heads 18. Proliferative retinopathy 19. Leg ulcers

Sickle Cell Disease

Diagnostic tests/findings include: 1. Hemoglobin - falls 2 standard deviations below mean for age and gender 2. Peripheral blood smear - hypochromic, microcytic red cells; MCV decreased by 2 3. Wide red cell distribution width \>17 4. Decreased serum ferritin (first to fall) 5. Decreased total serum iron 6. Increase iron-binding capacity 7. Decreased iron saturation 8. Elevated free erythrocyte protoporhyrin

Iron-Deficiency Anemia

Diagnostic tests/findings include: 1. CBC - generally the only required test 2. Platelet count

ITP

Signs and symptoms include: Great variations in frequency, severity, and bleeding manifestations 1. Nosebleeds 2. Bleeding gums 3. Heavy menstrual bleeding 4. Prolonged oozing from cuts 5. Increased bleeding after trauma or surgery

VWD

X-linked or autosomal recessive genetic disorder in which activity of red blood cell enzyme is decreased or absent, causing hemolytic anemia; most common among individuals of African, Mediterranean, or Asian descent

G6PD

Mild to moderate clinical severity with sickle hemoglobin 65-90%, hemoglobin A 5-30%, and fetal hemoglobin 2-20%; near normal lifespan; SB

Sickle Beta Thalassemia

Physical findings include: jaundice, cardiac murmur, splenomegaly, and pallor

SCD

Causes functional asplenia, leading to extreme susceptibility to infection. Pneumococcal immunization and PCN prophylaxis initiated for infection prevention.

Splenic Infarction

Physical findings include: 1. Mild - normal physical examination 2. Pallor 3. Tachycardia 4. Systolic murmur 5. Hepatomegaly 6. CHF

Iron-Deficiency Anemia

Management/treatment includes: 1. Factor VIII concentrate intravenously

Hemophilia A

Made in the bone marrow via fragmentation of megakaryocytes; circulate for 7-10 days.

Platelets

Physical findings include: 1. Easy bruising 2. Multiple sites of bruising 3. Oozing or bleeding at trauma or surgical site

VWD

Hemoglobinopathy associated with deletion of one or more alpha globin genes, increased but ineffective erythropoiesis, and shortened red cell life span

Alpha Thalassemia

Mild to moderate decrease in VWF

Type 1 VWD

Heme Flashcards

(94 cards)