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Pediatric Emergencies Exam 2 > Hematology > Flashcards

Hematology Flashcards

1
Q

Overview of ITP (7)

A
  1. Benign, acquired, isolated thrombocytopenia with platelets <100,000/ml
  2. May or may not present with bleeding
  3. Autoimmune disease without any other underlying disease process
  4. Secondary ITP is an autoimmune process occurring in response to another process in the body
  5. Peaks between ages 2 to 4 year old
  6. Equal gender distribution
  7. Increased risk for severity and chronicity with increasing age
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2
Q

Primary ITP Definition

A

Autoimmune disorder characterized by an isolated thrombocytopenia, platelet count <100,000/mL with no other identifiable cause for the thrombocytopenia

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3
Q

Secondary ITP Definition

A

All other forms of ITP not considered primary

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4
Q

Newly diagnostic ITP Definition

A

First 3 months after dx

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5
Q

Chronic ITP Definition

A

Lasting greater than 12 months after diagnosis

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6
Q

Severe ITP Definition

A

Presence of bleeding at diagnosis that requires intervention; presence of new bleeding symptoms that requires additional interventions with a different agent, or at an increase dose

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7
Q

Persistent ITP Definition

A

Between 3-12 months after dx

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8
Q

Common ITP Triggers for Primary ITP (6)

A
  1. Viral illness
  2. 72% newly diagnosed cases with history of recent viral illness
  3. Immunizations
  4. MMR
  5. Varicella
  6. Hepatitis A
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9
Q

Common ITP Triggers for Secondary ITP (6)

A

Chronic Infections

  1. H. pylori
  2. HIV
  3. Hepatitis C

History of Autoimmune disease in the child

  1. SLE
  2. Anti-phospholipid Syndrome
  3. Autoimmune neutropenia
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10
Q

ITP Pathophysiology (5)

A
  1. Platelet surface is covered with increased amounts of IgG
  2. Autoantibody-coated platelets induce Fc receptor- mediated phagocytosis by mononuclear macrophages, primarily but not exclusively in the spleen.
  3. The spleen is the key organ in the pathophysiology of ITP, Platelet autoantibodies are formed in the white pulp
  4. Mononuclear macrophages in the red pulp destroy immunoglobulin-coated platelets, removing affected platelets from circulation
  5. Platelet production is increased in bone marrow but can’t offset the destruction
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11
Q

ITP Clinical Presentation (9)

A
  1. Generally healthy child
    a. CBC is normal except for a low platelet count
    b. If everything is fine, presentation wise, CBC, etc, then they don’t get a bone marrow biopsy
    i. No indication of leukemia
  2. Acute onset of bleeding and bruising
  3. No history of fever, fatigue, or weight loss
  4. No family history of bleeding disorders
  5. Recent “cold” or live attenuated vaccination
  6. Bruising, petechiae or purpura
  7. Otherwise normal physical exam—NO
  8. HEPATOSPLENOMEGALY
  9. Mucosal bleeding usually indicates lower platelet count and worrisome
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12
Q

Dif Dx: Inherited bleeding disorders (4)

A

a. Wiskott-Aldrich Syndrome (small platelets)

b. vonWillebrand Disease - #1 disorder of coagulation
c. DiGeorge syndrome

d. Thrombocytopenia with Absent Radii syndrome

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13
Q

Dif Dx: bone marrow failure or infiltrates (3)

A

a. Intravascular Coagulation
b. Hemolytic-Uremic Syndrome
c. Thrombotic Thrombocytopenia

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14
Q

Dif Dx: Platelet activation and consumption (4)

A
  1. Disseminated Intravascular Coagulation
  2. Hemolytic-Uremic Syndrome
  3. Thrombotic Thrombocytopenia
  4. Kasabach-Merritt syndrome
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15
Q

Dif Dx: Immune mediated (4)

A
  1. ITP
  2. Drug-induced
  3. Systemic Lupus Erythematous
  4. Evans Syndrome (presence of simultaneous or sequential direct Coombs-positive autoimmune hemolytic anemia (AIHA) in conjunction with immune- mediated thrombocytopenia, with no known underlying etiology)
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16
Q

ITP Diagnostics (5)

A
  1. CBC with peripheral smear
    a. Platelet count <100,000/mL
    b. Isolated thrombocytopenia,
    c. Other cell lines are normal
    d. Large, well granulated platelets on peripheral smear –
    Look at MPV and it will be large
  2. Type and Screen
  3. Direct Antiglobulin Test, Direct Coombs Test; Direct Anti-human Globulin Test
    a. Sign of hemolysis?
  4. Bone Marrow?
    a. Not usually done if everything is normal
  5. In general you will refer to hematology for further management
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17
Q

First line recommendations for ITP (3)

A
  1. Observation + bleeding precautions IVIG
  2. Corticosteroids
  3. Anti-D Immunoglobulin
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18
Q

Second line recommendations for ITP (2)

A
  1. Rituximab

2. TPO-R agonists

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19
Q

Third line recommendations for ITP (4)

A
  1. Splenectomy
  2. Mycophenolate (cellcept)
  3. Azathioprine
  4. Vincristine
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20
Q

High risk of chronic ITP (7)

A
  1. Female gender
  2. Age ≥11 years at presentation
  3. No preceding infection or vaccination
  4. Insidious onset
  5. Platelet count ≥20 × 10 9/L at presentation
  6. Presence of antinuclear antibodies
  7. Treatment with methylprednisolone plus intravenous immunoglobulin
21
Q

First line therapies for ITP: Observation + Thrombocytopenic Precautions (5)

A
  1. No contact sports or gymnastics; discourage bike riding, skateboarding, rollerblading, etc.
  2. Toddlers need extra supervision
  3. Soft toothbrush
  4. Monitor for bruising, blood in mouth, urine, and stool.
  5. No treatments for platelets of 90,000 or more
22
Q

First line therapies for ITP: Corticosteroids (2)

A
  1. For initial (induction) treatment, in patients with a platelet count of 20-30 × 109/L [20-30 × 103/μL] and/or mucocutaneous bleeding), one regimen is prednisone 4-8 mg/kg/d with the intent of a rapid and complete taper after 7-10 days or when the platelet count reaches 50 × 109/L (50 × 103/μL), whichever occurs first.
  2. IV infusion of a corticosteroid may be preferable in critical situations
23
Q

First line therapies for ITP: IV RhIG (4)

A
  1. Competitive interference with Fc receptor macrophages in the spleen by coating Rh+ red blood cells
  2. Rapid rise in platelets (1-2 days)
  3. Black box warning and risk for massive hemolysis ▪ Rh+ patient
  4. Compared with IV RhIG, IVIG is associated with more adverse effects, longer infusions, and increased cost, causing many hematologists to prefer IV RhIG as a supplement to corticosteroids, at least for Rh(D)-positive patients.
24
Q

First line therapies for ITP: IVIG (6)

A
  1. Binds to Fc receptor on macrophage preventing circulating platelet destruction
  2. Rapid rise in platelets (1-2 days)
  3. Used in Rh Negative patients
  4. Expensive
  5. Infusion reactions
  6. immune problems/increased risk of infection with low platelet count
25
Q

Second line therapies for ITP (3)

A
  1. Rituximab
  2. MOA
    i. Monoclonal antibody that targets B cells to disrupt antibody production
    b. Infusion reactions and increased risk for infection
  3. TPO-R Agonists
    a. Romiplostim
    b. Eltrombopag
    i. Both increase platelet production
    ii. Safe and effective, need more studies
26
Q

Third line therapies for ITP (2)

A
  1. Splenectomy
    a. Only curative therapy
    b. Not recommended before 12 months of disease d/t high rate of remission
    c. Most providers prefer medical management vs. surgical
  2. Chemotherapy and Immunosuppressant
    a. Needs more research
    b. Used for chronic disease and ITP not responsive to other therapies
27
Q

ITP Summary (3)

A
  1. ITP is a diagnosis of exclusion
  2. Refer to nearby specialist if possible, not the ER
  3. Treat the clinical picture not just the numbers
28
Q

Overview of hemophilia (4 with info)

A
  1. X-linked recessive disorder of coagulation
    a. Deficiency of factor VIII is hemophilia A
    b. Deficiency of Factor IX is hemophilia B (one in 5000 men)*
  2. Acquired Hemophilia occurs when antibodies to these clotting factors form and block their function
    a. The percentage of the factor missing determines the severity of the disease
    b. Antibodies are attacking the clotting factors
  3. Hemophilia can be
    a. Mild: 6-40% of factor present
    b. Moderate: 2 to 5% implies moderate disease with bleeding present following minor trauma
    c. Severe: Less than 1% is severe disease with proclivity to spontaneous hemorrhage
    d. 2/3 of male patients have severe disease
  4. About 7 of 10 people with hemophilia A have the severe form.
    a. Normal persons have factor VIII activity of 100 percent
29
Q

Early presentation of hemophilia (3)

A
  1. Children with very mild hemophilia may not have noticeable symptoms for a few years. Often, the first sign is heavy bleeding from a dental procedure, an accident or surgery.
  2. Children with mild to moderate hemophilia may not have noticeable signs or symptoms at birth
  3. Males with severe hemophilia may bleed after circumcision.
30
Q

Early signs of hemophilia in young infants (3)

A
  1. Heavy bruising and bleeding from the gums as they cut their baby teeth
  2. Bumps and bruises from frequent falls as they learn to walk
  3. Swelling and bruises from bleeding in the joints, soft tissue and muscles
31
Q

Early signs of hemophilia in older children and adults (6)

A
  1. Acute Hemarthrosis
  2. Soft tissue and muscles hematoma
  3. Excessive bleeding in the mouth from laceration or tooth loss
  4. Spontaenous epistaxis
  5. Blood in the urine
  6. Blood in the stool
32
Q

Hemophilia clinical presentation: history of (5)

A
  1. Bruising
  2. Hemarthroses
  3. Intramuscular hematomas
  4. Intracranial hemorrhage is less common
  5. Initial presentation may not be dramatic
33
Q

Diagnostic Labs in Hemophilia (4)

A
  1. The CBC will have a normal platelet count
  2. If acutely bleeding, the child will have an acute normocytic anemia
  3. The Prothrombin time or (PT) will be normal
  4. The partial thromboplastin time is prolonged
34
Q

Major Signs in Hemophilia (4)

A
  1. Depends on the severity and type of hemophilia
    a. Bleeding
    b. Bruising
  2. Internal bleeding is common in people with severe hemophilia
  3. Internal bleeding can lead to damaged joints, muscles, or other parts of the body so prompt treatment is essential
  4. Compartment syndrome
    a. Faschia restricts the muscle and that cuts off the blood supply
35
Q

Acute hemarthrosis (8)

A
  1. Pain
  2. Swelling
  3. New onset limp
    a. In pain and they’re too young to express it
  4. Limitation of ROM
    a. Muscle atrophy
  5. Provokes a strong synovial inflammation leading to cartilage erosion, synovial hypertrophy and friability
    a. One indication for immediate ENT referral is a nasal hematoma because the cartilage gets destroyed
  6. Muscle atrophy around joint leads in instability increases risk of further hemarthrosis
  7. Poorly treated leads to cartilage destruction and osteoarthritis
  8. Joint aspiration not recommended
36
Q

Acute hemarthrosis treatment (10)

A
  1. Splinting
  2. Ice
  3. Immobilization
  4. Elastic bandages
  5. Analgesia
  6. Single factor infusion to raise level to 30% to 50% of normal
  7. In a joint with a history of multiple hemarthrosis, may need more than one infusion
  8. Range of motion and physical therapy
  9. Hip joint bleeding can lead to aseptic necrosis of the femoral head
  10. Factor replacement must be to 80% to 100% with daily replacement of 50%
37
Q

Treatment of Intramuscular Bleeding: Large weight bearing muscles (4)

A
  1. Slow bleeding over extended period
  2. Factor replacement of 30% to 50% of normal
  3. Can result in compartment syndrome especially if in the calf, forearm and hand
  4. Nerve paralysis or vascular compromise can occur
38
Q

Intramuscular bleeding: Iliopsoas hemorrhage: Characteristic triad, diagnosis and management

A
  1. Pain from massive hemorrhage
  2. Paresthesia along the anterior thigh from femoral nerve compression
  3. Flexion of the thigh
  4. Diagnostic test: ultrasound
  5. Management: Admit and replace with factor levels to 80% to 100%
39
Q

Intracranial Bleeding symptoms (5)

A

a. Headache
b. Lethargy
c. LOC
d. Vomiting
e. Seizure

40
Q

Treatment of Intracranial Bleeding (3)

A
  1. Infuse with factor to 100%
  2. No delay of infusion with factor until imaging studies are done
  3. Any child with hemophilia and a blow to the head must be treated with factor replacement immediately
    * Admit for observation
41
Q

Other Situations Requiring Factor Replacement (6)

A
  1. Laceration repair
  2. Lumbar puncture
  3. Surgery
  4. Dental extraction
  5. Men can have hematuria
  6. IM injections, Aspirin, jugular and femoral venipunctures should be avoided
42
Q

Treatment options for hemophilia (3 w/ info)

A
  1. Replacement therapy
    a. Replacing the clotting factor that is too low or missing.
    b. Concentrates of the clotting factor are infused IV
  2. Specific factors used to treat hemophilia are:
    a. Factor VIII for hemophilia A
    b. Factor IX for hemophilia B
  3. Replacement therapy can be used
    a. To prevent bleeding (prophylactic or preventive therapy)
    b. To stop bleeding when it occurs, on an as needed basis (demand therapy).
43
Q

Indications for Kovaltry (4)

A
  1. Hemophilia A (congenital Factor VIII deficiency)
  2. On-demand treatment and control of bleeding episodes
  3. Perioperative management of bleeding
  4. Routine prophylaxis to reduce the frequency of bleeding episodes
44
Q

What is Kovaltry (3)

A
  1. Recombinant, human DNA sequence derived, full length Factor VIII concentrate.
  2. Temporarily replaces the missing clotting Factor VIII that is needed for effective hemostasis.
  3. Kovaltry is supplied as a powder for solution for intravenous use after reconstitution only. Control of bleeding episodes and perioperative management:
45
Q

Kovaltry Dosing (2)

A
  1. Required dose (IU) = body weight (kg) x desired Factor VIII rise (% of normal or IU/dL) x reciprocal of expected/observed recovery (e.g., 0.5 for a recovery of 2 IU/dL per IU/kg).
  2. Estimated Increment of Factor VIII (IU/dL or % of normal) = [Total Dose (IU)/body weight (kg)] x 2 (IU/dL per IU/kg).
46
Q

Kovaltry Prophylaxis (2)

A
  1. Adults and adolescents: 20-40 IU/kg 2 or 3 times per week

2. Children ≤12 years old: 25-50 IU/kg 2 times per week, 3 times per week or every other day

47
Q

Treatment with DDAVP in Hemophilia A: Mild Hemophilia (3)

A
  1. Replacement therapy is usually not needed for mild hemophilia; however, a medication called desmopressin (DDAVP) is sometimes given to raise the body’s levels of factor VIII
  2. Since the effects wear off with chronic use, it is applied only in certain situations (for ex: prior to dental work or participation in sports) to prevent or reduce bleeding.
  3. DDAVP does NOT help in hemophilia B
    a. Only with hemophilia A
48
Q

Hemophilia: Problems with Treatments and Alternatives (6)

A
  1. Approximately 30% of patients with severe factor VIII deficiency will develop an inhibitory IgG antibody against the infused factor VIII
  2. Alternatives include Recombinant factor VII (short half life and needs to be given every 2 hours) and prothrombin complexes (bypass the need for factor VIII due to presence of factors II, VII, and X
  3. Amicar (epsilon aminocapric acid) or Cyklokapron (tranexamic acid) are clot stabilizers which can be used for prevention or treatment or oral hemorrhage
  4. Oral or IV
  5. DDAVP increases factor VIII levels in patients who have mild hemophilia
  6. IV over 30 minutes or intranasal

Pediatric Emergencies Exam 2 (9 decks)

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