8 - Pediatric Hematology-Oncology 2026

8-12 weeks

Physiologic anemia of infancy usually lasts for how long?

Switch from fetal to adult Hb -> downregulation of EPO production

Central cause of physiologic anemia of infancy?

7-9 g/dL

Physiologic anemia of prematurity has nadir levels of?

EPO

RBC transfusion

Management of anemia of prematurity?

T.A.I.L.S.

• Thalassemia

• Anemia of chronic disease

• Iron deficiency

• Lead poisoning

• Sideroblastic anemia

Common microcytic anemias

H.U.B.A

• Hypothyroidism

• Uremia

• Bone marrow failure (aplastic anemia)

• Anemia of chronic disease

Common normocytic anemias

Folate deficiency

Vit B12 deficiency

Drug/alcohol-induced

Common macrocytic anemias

Iron deficiency anemia

Most common hematologic disease of infancy and childhood

Pallor

Most important sign of anemia that usually manifests when Hb drops to 7-8 g/dL

Iron Deficiency Anemia

A child with pallor presents with laboratory findings of microcytic and hypochromic RBCs, low reticulocyte count, and high RDW. Impression?

Thalassemia

A child with pallor presents with laboratory findings of microcytic and hypochromic RBCs, low reticulocyte count, and normal RDW. Impression?

Low serum iron

Low serum ferritin

High TIBC

Characteristic iron studies findings in IDA?

IDA has HIGH TIBC

ACD has LOW TIBC

What differentiates IDA from anemia of chronic disease based on iron studies?

Elemental Fe 3-6 mkDay in 2-3 divided doses x 2-3 months

Management of IDA?

0.1 – 0.4 g/dL/day

Expected rise in Hb during treatment?

48-72 hours

Reticulocytosis is a response expected within how many hours from Iron replacement therapy?

36-48 hours

When does one expect initial bone marrow response during iron therapy?

12-24 hours

Subjective improvement is noted within how many hours after receiving iron therapy?

4-30 days

Increase in Hgb level is expected within hour many days from starting iron therapy?

1-3 months

Repletion of iron stores happens within how many months from iron replacement therapy?

Thalassemia

Results when 1 or more globin genes mutates leading to microcytic hemolytic anemia

ALPHA thalassemia

Results when there is a decrease in ALPHA-globin chain synthesis

Thalassemia trait

When 2/4 foci are deleted/mutated, alpha thalassemia presents with mild anemia and is called?

Hemoglobin H disease

When 3/4 foci are deleted/mutated, alpha thalassemia presents with splenomegaly and increased B4 and is called?

Hydrops fetalis

4/4 foci deleted/mutated in alpha thalassemia is incompatible with life. This leads to?

1

Silent/asymptomatic alpha thalassemia has how many focus/foci affected?

Transfusions, chelation therapy

Splenectomy (as needed)

Management of alpha thalassemia includes

BETA thalassemia

Occurs when there is a decrease in BETA-globin chain synthesis or absence of beta chain of hemoglobin

Beta-thalassemia minor

Asymptomatic carriers in B-thalassemia with mild or no anemia?

Hb electrophoresis (increased HbF and HbA2)

Definitive diagnosis of B-thalassemia

Beta-thalassemia major (Cooley anemia)

Type of Beta-thalassemia characterized by NO beta-globin production

Transfusions, chelation therapy as needed,

hydroxyurea, splenectomy

(to treat the resultant hypersplenism)

Management of Beta-thalassemia

Spherocytosis

Hemolytic anemia resulting from defect in membrane spectrin or ankyrin (erythrocyte skeletal proteins)

Osmotic fragility test

Confirmatory blood test to establish presence of fragile sphere-shaped RBCs in spherocytosis?

Splenectomy

Folate supplementation

Treatment of spherocytosis

Sicke cell disease

An African-American child came in for anemia and severe lower extremity pain. Laboratory test confirms Howell-Jolly bodies on PBS

and Hb electrophoresis (HbS)

Autosomal recessive

Inheritance pattern of sickle cell anemia?

Crew-cut/hair-on-end apppearance on skull xray

Typical radiologic findings in sickle cell disease?

Hemophilia A

X-linked recessive deficiencies of factor VIII

Hemophilia B

X-linked recessive deficiencies of factor IX

von Willebrand disease

Most common hereditary

bleeding disorder?

Hemophilia A

Most common and most serious congenital coagulation factor deficiencies?

Factor V Leiden

Most common hereditary

hypercoagulable disorder?

Prolonged bleeding

Hallmark of hemophilia?

Desmopressin

Management of von Willebrand disease with mild bleeding?

Von Willebrand factor

A large protein made by endothelial cells and megakaryocytes; a carrier for factor VIII and is a cofactor for platelet adhesion.

Acute Lymphocytic Leukemia

Child presenting with acute signs anorexia, of bone marrow failure and organomegaly. Laboratory test shows pancytopenia and hypercellularity. Impression?

Aplastic anemia

Pancytopenia + hypocellularity on CBC. Impression?

Treatment

Single most important prognostic factor in ALL?

• 1. Age of the patient at the time of diagnosis

• 2. Initial leukocyte count

• 3Speed of response to treatment

Three of the most important PREDICTIVE factors in ALL

• < 1 yr or > 10 yrs

• Male

• WBC >50,000μ/L on presentation

• Presence of CNS leukemia

• Presence of a mediastinal mass

Poor prognostic factors in ALL?

Wilms Tumor

Renal tumor of embryonal origin and the 2nd most common malignant abdominal tumor in childhood?

• Neurofibromatosis

• Beckwith-Wiedemann syndrome

• WAGR syndrome

Conditions associated with Wilms tumor?

Wilms Tumor

A child 2-5 years old presenting with painless abdominal enlargement with flank mass that does not cross the midline, hematuria (12-25%), hypertension. Impression?

Transabdominal nephrectomy & post-surgical chemotherapy

Management of Wilms Tumor?

Neuroblastoma

A child coming in for non-tender abdominal mass which may cross the midline, Horner syndrome, hypertension. Impression?

VMA & HVA

Which enzyme in the urine is elevated in 95% of cases of Neuroblastoma?

Age at diagnosis, stage of disease, Shimada

histology

Prognostic factors for neuroblastoma?

Stage III

Neuroblastoma with tumor that extends beyond midline with or w/o bilateral lymph node involvement?

55-60%

83% for infants

55-60% for 1-5 yrs old

40% for children >5 yrs old

A 3 y/o child diagnosed with Neuroblastoma has a 5-year survival rate of?

Surgery

Management for St 1 and 2 Neuroblastoma

Observation

Management for St 4s neuroblastoma

Stage 4S

Neuroblastoma in <1 year old w/ dissemination to liver, skin, or BM w/o bone

involvement & with a primary tumor is tagged as?

Surgery + chemotherapy

Management for safe 3 & 4 neuroblastoma?

Ann Arbor Staging Classification

Staging used to classify lymphomas

Hodgkin Lymphoma

A child with adenopathy and B symptoms underwent biopsy that revealed Reed-Sternberg cells. Impression?

Non-Hodgkin Lymphoma

Lymphoma presenting with more extranodal involvement and hepatosplenomegaly?

Fanconi Anemia

Aplastic anemia with microcephaly, microphthalmia, hearing

loss, limb anomalies

Rhabdomyosarcoma

Most common soft tissue tumor?

PNET (Brain tumor)

Childhood malignancy with highest mortality?

Osteosarcoma

Malignancy involving the metaphysis of long bones and usually presents with sunburst pattern on imaging

Ewing sarcoma

Malignancy involving the diaphysis of long bones and presents as onion-skinning/moth-eaten appearance on imaging