Blood Chap 446-490 Flashcards by Karen Morales

Reduction of the hemoglobin concentration on the RBC volume below the range of values occurring in healthy persons

Anemia

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Hemoglobin level wherein clinical findings of pallor, sleepiness, irritability and decreased exercise tolerance

Hgb <7-8g/dl

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Most useful indicator of iron stores

Serum ferritin

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Reliable but late indicator of iron deficiency

Mean corpuscular volume (MCV)

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Rare, congenital bone marrow failure syndrome, symptomatic in early infancy
Associated with craniofacial abnormalities (hypertelorism, cleft palate), skeletal anomalies, noteable ABSENT RADIAL PULSE
MACROCYTIC anemia
Elevated Erythrocyte adenine deaminase activity, low reticulocyte

Dx & Tx?

Congenital Hypoplastic Anemia (Diamond-Blackfan Anemia)

Tx: Corticosteroid
*if unresponsive- hematopoietic stem cell transplantation

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MC ACQUIRED red cell aplasia

Transient Erythroblastopenia of Childhood (TEC)

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Severe, hypoplastic anemia in a previously healthy 1 year old boy
Noted to have a VIRAL ILLNESS prior to anemia

Normal MCV (Normocytic), with thrombocytosis
RBC adenosine deaminase is normal

Recovered after 2mos

Dx?

Transient Erythroblastopenia of childhood (TEC)

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Usual VIRAL cause of red cell aplasia in patients with chronic hemolysis, who are immunocompromised, and fetus in utero

Parvovirus B19 (causes erythema infectiosum/fifth disease)

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Brief cessation of erythropoiesis in patients with hemolysis causing severe anemia

Aplastic crisis

*Parvovirus induced aplastic crisis usually occurs only once

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Low serum iron
Low to normal serum ferritin

Anemia of chronic disease

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Site of erythropoietin production in fetus

Liver

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Site of erythropoietin production in infants

Kidney

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Progressive decline in Hgb in infants that starts during the first week of life and persists for 6-8wks, with major during 8 & 12 weeks

Physiologic anemia of infancy

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MC cause of folate deficiency in older children

Malnutrition

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Anemia in folate deficiency

Megaloblastic anemia

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T/F chronic diarrhea may cause folic acid deficiency

True, it disrupts enterohepatic circulation of folate enhancing loss

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T/F chronic diarrhea may cause folic acid deficiency

True, it disrupts enterohepatic circulation of folate enhancing loss

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Vegetarian or vegan diet

Vitamin B12 (Cobalamin) deficiency

*Megaloblastic anemia

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Excessive excretion of methylmalonic acid in the urine (0.3-5mg/24h)

Vitamin B12 deficiency (Cobalamin)

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Most important clinical sign of iron deficiency

Pallor

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Pallor is visible at Hgb of?

7-8g/dL

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Desire to invest nonnutritive substances

Pica

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Serum ferritin is
A. Decreased
B. Normal
C. Increased

in what type of anemia

A. IDA
B. THALASSEMIA
C. CHRONIC DISEASE

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Estimate of the body’s iron stores in the absence of inflammation

Serum Ferritin

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⬇️ serum ferritin ⬆️ serum transferrin Hypochromic, microcytic

Iron deficiency anemia

Regular response of iron deficiency anemia to oral iron

Increase in Hgb >/=1g/dL after one month

Therapeutic dose of elemental iron for IDA

3-6mg/kg in 3 divided doses max 150-200mg daily

IDA response to oral iron after A. 12-24 hrs B. 36-48 hrs C. 48-72 hrs D. 4-30 days E. 1-3 mos

A. Improvement of symptoms (subjective); decreased irritability, increased appetite; repletion of intracellular iron enzymes B. Erythroid hyperplasia; initial bone marrow response C. Reticulocytosis, peaking at 5-7 days D. Increase in hemoglobin level E. Repletion of stores

Premature destruction of RBC

Hemolysis

Laboratory evidence when seen denotes inravascular hemolysis

Free hemoglobin in plasma (plasma appears pink after centrifugation)

MC abnormality of the RBC membrane

Hereditary spherocytosis

Evidence of hemolysis

Reticulocytosis Indirect hyperbilirubinemia

MC inherited abnormality of RBC membrane

Hereditary Spherocytosis

Anemia, hyperbilirubinemia, splenomegaly Risk for GALLSTONE Reticulocytosis, spherocutyes on blood smear, inc MCHC

Hereditary Spherocytosis

Resistance to malarial infection

Hereditary Elliptocytosis

Extreme microcytosis Extraordinary variation of cell size & shape INCREASED THERMAL INSTABILITY

Hereditary pyropoikilocytosis

Fever is a medical emergency in this hemoglobinopathy

Sickle cell anemia *high risk for infection with encapsulated organisms due to functional asplenia

Life-threatening complication of Sickle cell anemia

Acute splenic sequestration

Left sided abdominal pain, rapid spleen enlargement, decline in hemoglobin at least 2g/dl Reticulocytosis Thrombocytopenia History of fever, bacteremia, or viral infection

Acute splenic sequestration

First manifestation of pain in infants & young children with Sickle cell anemia

Dactylitis (hand-foot syndrome) *unilateral or symmetric swelling of the hands &/or feet

Cardinal feature of Sickle cell anemia

Acute vasoocclusive pain

Cause of osteomyelitis in children with sickle cell anemia

S. Aureus Salmonella

Only drug proven effective in reducing the frequency of painful episodes in Sickle cell anemia

Hydroxyurea

Renal cell carcinoma is associated with

Sickle cell trait

T/F: 70% Methemoglobin is lethal

True

Blood is chocolate brown even when exposed to oxygen

Methemoglobin

Treatment to reduce Methemoglobin in Hereditary Methemoglobinemia

Ascorbic acid (200-500 mkday)

Treatment for Toxic Methemoglobinemia

Methylene blue (initially 1-2mg/kg IV, then 100-300mg PO as maintenance) *except for G6PD (ineffective)

Quantity of globin chains produced

Thalassemia

Quality of globin chains produced

Sickle cell disease

Maxillary hyperplasia, flat nasal bridge frontal bossing Hepatosplenomegaly

Thalassemic facies

Best indicator of total body iron stores

Quantitative liver iron

Major cause of death in thalassemia

Cardiac disease

Persistently low RDW and low MCV despite iron treatment

Beta thalassemia trait

Heinz bodies Polychromasia Bite cells

G6PD

Hallmark of autoimmune hemolytic anemia

Positive Direct Coomb's test

MC Cause of drug immune hemolytic anemia

Cephalosphorins

Autoimmune hemolytic anemia with concomitant ITP

Evans syndrome

Treatment for autoimmune hemolytic anemia

Glucocorticoids (Prednisone 2mkday up to 6 mkday) IVIG if severe despite steroids Rituximab in chronic refractory cases

Warm antibodies

IgG

Cold antibodies

IgM

Below normal values of 3 blood cell lineages

Pancytopenia

MC inherited Pancytopenia

Fanconi Anemia

Short stature Skin pigment changes (with café au lait spots( Upper limb abnormalities (absent of radii and thumbs, radial pulse is weak or bsent) Hypogonadal and genital changes Facies: microcephaly, small eyes, epicanthal folds Kidney abnormalities PROPENSITY FOR CANCER

Fanconi Anemia

MC cancer associated with Fanconi Anemia

Squamous cell carcinoma

Inherited pancytopenia with associated exocrine pancreas insufficiency (fat malabsorption) and skeletal abnormalities (metaphyseal dysplasia)

Shwachman-Diamond syndrome

Mucocutaneous (ectodermal) triad of Dyskeratosis Congenita

Reticulate skin pigmentation of the upper body Nail dystrophy Mucosal leukoplakia

Factor VIII deficiency

Hemophilia A

Factor IX deficiency

Hemophilia A

Measures the activation of factor X by factor VII

Prothrombin time *thus NORMAL in Hemophilia (factor VIII & IX deficiency)

Hallmark of hemophilic bleeding

Hemarthroses - bleeding in the joint

Vague area of referred pain in the groin Hip is held flex, internally rotated position with inability to extend hip

Ilipsoas muscle bleeding *may lead to hypovolemic shock

NORMAL Prothrombin Prolonged PTT

Hemophilia

Standard of care for severe hemophilia

Prophylaxis *Factor VIII (A) - 20-40 IU/kg every other day *Factor IX (B) - 30-50 IU/kg every 2-3 days

Failure of a bleeding episode to respond to appropriate replacement is the first sign of an

Inhibitor - antibodies directed against factor VIII or IX that blocks the clotting activity

Off-label medication used as alternative therapy for patients with high inhibitor titers in whom immune tolerance program have failed

Rituximab

MC inherited bleeding disorder

Von Willebrand disease

Presents with mucosal bleeding (+) family history of bleeding

Von Willebrand disease

MC type of VWD *presents with mucosal bleeding

Type I

Most severe type of VWD

Type 3 *may present with joint bleed or CNS hemorrhage

MC spontaneous thromboembolic event in neonates

Renal vein thrombosis

Reverse effect of heparin

Protamine sulfate

Rat poison

Vitamin K deficiency

MCC of acute onset thrombocytopenia in a well child

Idiopathic Thrombocytopenic Purpura (ITP)

MCC of acute onset thrombocytopenia

Idiopathic Thrombocytopenic Purpura (ITP)

Previously healthy 1 - 4yr old Sudden onset of generalized petechiae & purpura History of vital illness Profound thrombocytopenia in otherwise normal CBC

Idiopathic Thrombocytopenic Purpura (ITP)

Acute ITP with persistent thrombocytopenia for >12mos

Chronic ITP

Pentad of fever, microangiopathic hemolytic anemia, thrombocytopenia, abnormal renal function and CNS changes

Thrombotic Thrombocytopenic Purpura

Pentad of fever, microangiopathic hemolytic anemia, thrombocytopenia, abnormal renal function and CNS changes

Thrombotic Thrombocytopenic Purpura

Treatment for Thrombotic Thrombocytopenic Purpura (TTP)

Plasmapheresis