Pediatric Hematology Quiz

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Questions and Answers

Which medication inhibits clot destruction during bleeding disorders?

NSAIDs

Desmopressin

Amino-caproic acid (correct)

Factor VIII infusion

What is NOT a sign of bleeding that should be monitored?

Hematuria

Tarry stools

Excessive thirst (correct)

Joint stiffness

Which of the following statements about Sickle Cell Disease (SCD) is correct?

SCD only affects individuals of African descent.

Median life expectancy for individuals with SCD is now nearly 95 years.

RBCs typically lose their shape in high oxygen environments.

Many families keep factor infusions for bleeding emergencies at home. (correct)

Which of the following is a characteristic of Sickle Cell Disease?

Acute and Chronic Organ Damage Signup and view all the answers

What impact does Desmopressin have in treating bleeding disorders?

It increases plasma factor VIII levels. Signup and view all the answers

What defines severe neutropenia?

ANC < 500 Signup and view all the answers

Which area is NOT typically considered a site of infection risk in pediatric oncology patients?

Feet Signup and view all the answers

Why is oral hygiene particularly important for patients with mucositis?

Mucositis can increase the risk of infection in the mouth. Signup and view all the answers

What is a common mental health challenge faced by pediatric cancer survivors?

Body image concerns due to alopecia Signup and view all the answers

What might be a consideration for preserving fertility in adolescent cancer patients?

Egg harvesting procedures if applicable Signup and view all the answers

Which symptom is often experienced during vaso-occlusive episodes in individuals with SCD?

Severe pain Signup and view all the answers

What is a common trigger for vaso-occlusive episodes in sickle cell disease?

Dehydration Signup and view all the answers

Which condition is characterized by sickled cells blocking blood flow to the lungs?

Acute Chest Syndrome Signup and view all the answers

What increases the risk of stroke in children with SCD?

Sickled red blood cells blocking vessels Signup and view all the answers

What is the management recommendation for individuals with SCD experiencing complications?

Hydration Signup and view all the answers

Which of the following is a potential consequence of untreated priapism in SCD?

Erectile dysfunction Signup and view all the answers

Functional asplenia in pediatric patients with SCD can lead to which serious health risk?

Higher risk of blood infections Signup and view all the answers

What symptom should individuals with SCD seek emergency care for?

Fever Signup and view all the answers

What is the primary treatment for Beta-Thalassemia Major?

Blood stem cell transplant Signup and view all the answers

Which factor can contribute to iron deficiency anemia in adolescents?

Poor dietary intake of iron Signup and view all the answers

What should be avoided when administering iron supplements to increase absorption?

Milk products Signup and view all the answers

Which of the following is a common lab finding for iron deficiency anemia?

Decreased RBC and Hgb counts Signup and view all the answers

What symptom is frequently associated with chronic iron deficiency anemia?

Pica (cravings for non-nutritive substances) Signup and view all the answers

What is the role of cryoprecipitate in blood transfusions?

To help with clotting Signup and view all the answers

Which condition may require an RBC infusion to prevent congestive heart failure?

Severe anemia Signup and view all the answers

What is a common dietary recommendation for older children at risk of iron deficiency?

Red meat and poultry Signup and view all the answers

What characteristics define the preferred blood products for oncology patients?

Leuko-reduced and CMV-negative Signup and view all the answers

Which lab tests are crucial for monitoring Tumor Lysis Syndrome?

Potassium, Uric Acid, and Phosphate Signup and view all the answers

What happens to infants born with Severe Combined Immunodeficiency (SCIDs)?

They are susceptible to severe infections. Signup and view all the answers

What is the most common type of cancer associated with a mediastinal mass in pediatric patients?

Malignant lymphomas Signup and view all the answers

What is the typical age for the development of osteosarcoma in children?

After 10 years of age Signup and view all the answers

What is a common treatment to mitigate the risk of Tumor Lysis Syndrome?

Allopurinol administration Signup and view all the answers

Which is NOT a symptom of Functional Neutropenia?

Normal immune response Signup and view all the answers

What was David Vetter famously known for during his life with SCIDs?

Living in a germ-free plastic bubble Signup and view all the answers

What is the typical age range for diagnosing rhabdomyosarcoma in children?

Under 6 years Signup and view all the answers

Which of the following is a characteristic symptom of neuroblastoma?

Sympathetic nervous system involvement Signup and view all the answers

What is the most common treatment approach for Wilms tumor?

Surgical removal of the tumor Signup and view all the answers

What is the median survival time for patients with diffuse intrinsic pontine glioma (DIPG)?

10-11 months Signup and view all the answers

Which of the following treatments is NOT typically used for high-risk neuroblastoma?

Magnet therapy Signup and view all the answers

In which anatomical location are osteosarcomas most often found?

Long bones of the legs and arms Signup and view all the answers

What is a potential risk when palpating the abdomen of a patient suspected of having Wilms tumor?

Rupture of the renal capsule Signup and view all the answers

What has been the trend in the 5-year survival rate for patients with rhabdomyosarcoma from 1975 to 2017?

Increased gradually Signup and view all the answers

Study Notes

Pediatric Hematology Oncology

This presentation covers various pediatric blood disorders including treatments and management.
The speaker's pronouns are she/they.
The date of the presentation is May 7th, 2024.
The presentation title is Pediatric Hematology Oncology.

Hemophilia

Hemophilia is a group of bleeding disorders.
Characterized by difficulty controlling bleeding due to deficiencies in clotting factors.
Hemophilia A accounts for 80% of cases.
Hemophilia B is due to a deficiency in factor IX.
Bleeding time is extended, potentially internal or external.
Bleeding disorders can be recognized in infancy.
Sometimes characterized by petechial or purpuric rashes.
Unexpected bruising in young children, especially under 6 months old, can be a sign.
Individuals who don't cruise (crawl) rarely bruise as a sign of clotting problems.

Purpura & Petechiae

Purpura and petechiae are characterized by spots that do not blanch.

Hemophilia A & B

Both A and B are X-linked recessive disorders, meaning they are linked to the X chromosome.
Tests for hemophilia may include PT/INR and aPTT.

Von Willebrand Disease

Von Willebrand disease is due to a lack of Von Willebrand Factor protein.
Platelets have an impaired ability to aggregate.
PTT and PT/INR tests may be within normal limits (WNL) for these patients.
Von Willebrand Factor Antigen and Activity tests may be used for diagnosis.

Management of Bleeding Disorders

Controlling bleeding is a primary management strategy.
Avoiding aspirin and NSAIDs is often recommended, unless part of replacement therapy due to possible interference with healing.
Minimizing pokes (invasive procedures) when possible is important.
Close monitoring for bleeding symptoms is essential, including active bleeding, hematomas/bruising, joint pain and stiffness, etc.

Bleeding Assessments

Active bleeding (gums, nosebleeds, blood in urine, black/tarry stools)
Hematomas (bruises)
Hemarthrosis (joint pain and stiffness with swelling)
Neuro assessment (headache changes, slurred speech, and lethargy)
Vital signs and pain assessment
Asking caregivers what's abnormal is recommended.

Medications for Bleeding Disorders

Antifibrinolytics, such as Amino-caproic acid (Amicar), are used to inhibit clot destruction, enabling more clotting.
Medications such as DDAVP (Desmopressin), a synthetic form of vasopressin increase plasma factor VIII.
Factor VIII or pooled plasma (IV infusion) increases clotting factors.
Some families may have clotting factor at home for emergencies.

Sickle Cell Disease

The world's most common heritable blood disorder.
Misperceptions exist regarding impacting solely Black Americans.
Red blood cells under hypoxic conditions form "sickle" shapes.
The median life expectancy of patients with SCD in the US (1994) was 42-47 years, but now is approaching 95 with age 18.
Typically milder (HbSC, HbS beta thalassemia) and typically more severe (HbSS) forms exist.
Included in Newborn Screening.

CDC Facts on Sickle Cell Disease

A child gets sickle cell disease from inheriting two sickle cell genes (one from each parent).
A child with only one sickle cell gene has sickle cell trait.
Bone marrow transplant can cure some patients, but is risky.
Anemia commonly associated with sickle cell disease can be treated with blood transfusions.
Individuals with SCD can enjoy a long and high-quality life with regular checkups, treatments (like hydroxyurea), and protective measures to prevent infections.

Sickle Cell Disease Characteristics

Anemia and associated symptoms
Vaso-occlusive pain episodes (pain crises)
Chronic pain
Acute and chronic organ damage
Mental health sequelae (potential implications on mental health)
Increased susceptibility to infection

Vaso-Occlusive Episodes/Pain Crises

Characterized by severe pain, described as "multiple fractures" and "glass flowing through the body", and "like being stabbed repeatedly while having migraine throughout whole body"
Potential triggers include dehydration, temperature changes, stress, and infections.
High altitude is a potential trigger too.

Complications of SCD

Acute chest syndrome (blood and oxygen blocked in lungs)
Signs include fever, upper respiratory infection (URI) symptoms, chest pain, low oxygen saturation (SpO2).
Treatment may involve antibiotics, IV fluids, pain management, and incentive spirometry.
Priapism (prolonged, painful erection)
Stroke (blockage of blood flow to the brain)
Stroke risk increased by 100-fold in children with SCD as compared to children without.

Complications of SCD cont.

Splenic sequestration (sickled RBCs trapped in spleen, causing blood pooling and splenomegaly).
Under-functioning spleen=higher infection risk.
Infections are problematic for patients with SCD.
Fever is a potentially dangerous sign in patients with SCD, needing immediate medical care.

Management of Sickle Cell Disease

Hydration
Incentive spirometry
Collaborative pain management
Pain assessment
Continuous infusions
Multi-modal therapies (VR goggles, heat packs)
Antibiotics
Oxygen administration
Blood product administration, removing sickle cells and replacing with normal cells.

Big Takeaways for SCD

Believe and treat patient's pain, support oxygen requirements, prevent further hypoxic stress.

Big Medications for SCD

Prophylactic penicillin prevents pneumococcal infections, especially in younger children and those with asplenia (no spleen).
Tylenol and ibuprofen for mild-moderate pain, while opioids may be needed for moderate-severe pain.
Hydroxyurea increases fetal hemoglobin. It helps keep red blood cells round and increases oxygen-carrying capacity.

Cure? (Bone Marrow or Hematopoietic Stem Cell Transplant)

FDA-approved method for treating sickle cell disease
Most common in children with multiple complications
Well-matched donor is needed for best outcome of transplant
This area of research remains ever-growing.

FDA Approves First Gene-Editing Treatments for Human Illness

A gene-editing treatment for human illness has been approved by the FDA.
Victoria Gray was the first person in the US to receive it.

Bone Marrow Transplant

Bone Marrow transfusions involve transferring healthy bone marrow from a donor into a child with disease.
Transplant cells develop normally
Conditioning prep, may involve meds to weaken the immune system (immunosupprive)
Complicications include Graft vs Host Disease(GVHD), and Graft failure.

BMTS (Bone Marrow Transplant Successes)

Conditions treatable via BMTS

Odds of Finding a Match for Transplant (BMT)

Odds of patient finding a full match are not good based on the ethnicity of the given donors.

Thalassemia

Reduced hemoglobin production
Typically diagnosed in childhood
Often presenting with severe anemia, jaundice, delayed growth, and skeletal abnormalities.
Beta-thalassemia Major is the typically most severe form.

Iron Deficiency Anemia

Iron is required for hemoglobin production
Iron deficiency results in low hemoglobin levels
Risk factors include excessive cow's milk intake, premature birth, malabsorption disorders, and poor dietary iron intake.

Assessment of Anemia

Findings and symptoms in a patient with Anemia include tachycardia, pallor, brittle or clubbed fingernails, fatigue, heart irregularities; the patient may exhibit Pica (desire to consume non-food items.)
Lab results include a low count of RBCs, hemoglobin, and hematocrit results.

Management of Iron Deficiency Anemia

Iron supplementation, ideally in conjunction with vitamin C, for better absorption.
Avoid giving iron with milk, tea, or antacids as this decreases rate of absorption.
Give liquid iron through straws to prevent enamel staining.
Encourage kiddos to consume foods and formulas with iron.

Blood Transfusions

The process of transfusing blood into a patient
Used to treat or prevent several blood disorders.

Blood Products

Cryoprecipitate helps with clotting factors.
Plasma carries proteins, ions, and nutrients.
Platelets aid in clotting.
Red blood cells help with oxygen transport.

Blood Products (special Considerations for Hematology-Oncology patients)

Oncological patients may need leuco-reduced and CMV-negative blood products to avoid immune system problems.
In patients with Sickle Cell Anemia, hemoglobin S negative blood products may be required to further avoid problems with blood disorders.
Repeated blood transfusions may delay results in older children due to crossmatching issues.

Oncology Lab Considerations

Neutrophils are first responders in the immune system, can be low in patients with neutropenia.
Blasts can be a potential problem in patients with immunocompromise.
Patients may have blood issues like low neutrophils (ANC) despite being over the threshold.
Other lab testing required for hematological patients may involve measuring levels of Potassium, Urea, and Phosphate levels.
Monitor renal function in patients with hematological challenges.
Administering allopurinol might mitigate TLS risk, that is, to reduce the risk of tumor lysis syndrome.

Mediastinal Mass

A mass in the middle of the chest.
More common in malignant lymphomas.
Can compromise airway anatomy.

SCIDS (Severe Combined Immunodeficiency)

Rare disorder due to mutations in immune system development and function.
Infants appear healthy at birth, but they are highly susceptible to severe infections due to severe immuno-compromised status.
Bone Marrow Transplants are a crucial cure option.

Osteosarcoma

The most common bone cancer in children.
Typically develops after age 10.
Usually found in long bones (legs/arms)
Common locations include distal femur and proximal tibia.
Treatment usually involves chemotherapy and surgical removal.

Rotationplasty (part of Osteosarcoma treatment)

The surgical procedure is designed to reconstruct a part of the leg.

Rhabdomyosarcoma

It is a common soft tissue cancer in children, showing up in various parts of the body.
Two-thirds of cases are seen under the age of 6.
Multi-modal treatment is typical (surgical, chemotherapy, and possibly radiation).

Neuroblastoma

Rare form of cancer affecting the sympathetic nervous system in the adrenal glands, abdomen, and chest.
Usually present in children under age 5.
Treatment may include surgery, radiation, high-dose chemotherapy with stem cell transplant or monoclonal antibody therapy.

Diffuse Intrinsic Pontine Glioma (DIPG)

A tumor that impacts the pons, a part of the brain stem.
A tumor in the brain.
One of the most common types of pediatric brain tumors.
A multi-modal approach to therapy, but there is no known cure.
Median survival is 10-11 months.

Wilms' Tumor (Nephroblastoma)

Cancer of the kidney (or rare form occurring in the abdomen)
Typically seen between ages 2 and 3
Often diagnosed when unilateral
Should not be palpated, as this may further rupture the kidney
Aggressive procedures are often used for diagnosis and treatment.

Fever & Neutropenia (F&N)

Common emergency situation in immunocompromised pediatric patients who often have underlying blood issues.
Fever in neutropenic pediatric patients should be treated as an emergency.
Neutropenia is characterized by low neutrophil counts.
Mild, moderate, and severe neutropenia are graded based on ANC (absolute neutrophil count).
Parenteral antibiotics may be necessary in patients with neutropenia, especially with fever. This requires close monitoring by clinicians.

Infection Risk

Central line infections (CLABSIs) are a common concern in pediatric oncology patients,
Mouth care, and oral hygiene are important!
Diaper areas and skin that is compromised need careful monitoring.
Any places where skin is broken down need care to prevent infection.
Mucositis from chemotherapy requires close medical monitoring.

Mental health implications in cancer patients and survivors must be considered.
Fertility preservation should be discussed, offering egg or sperm preservation.
How alopecia (hair loss) impacts patients emotionally should also be considered.

Happy Studying!

The presentation ends with a message encouraging continued learning.

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Test your knowledge on key concepts related to pediatric hematology, including bleeding disorders, sickle cell disease, and the implications of treatments like Desmopressin. This quiz covers signs, symptoms, and considerations in managing these conditions, particularly in children with cancer. Perfect for students and healthcare professionals in the field of pediatrics.