ABP Pediatric Hematology-Oncology flashcards

32 cards. Read the term and the definition here. The deck at the top of this page is optional.

Mentzer index

MCV divided by RBC count; a value below 13 favors thalassemia trait, whereas a value above 13 favors iron deficiency, though pediatric age and other conditions limit accuracy.

Iron deficiency treatment

Oral elemental iron is commonly dosed at 3–6 mg/kg/day; continue several months after hemoglobin normalizes to replenish stores. Transfuse only for severe symptomatic anemia.

Warm autoimmune hemolytic anemia

Immune hemolysis with a positive DAT for IgG; first-line therapy is corticosteroids, and transfusion is given for symptomatic anemia using the least incompatible units rather than withheld absolutely.

Hydroxyurea in sickle cell

Daily hydroxyurea reduces painful episodes, acute chest syndrome, and transfusions in sickle cell disease; monitor CBC and adherence, and counsel about reversible myelosuppression and potential fertility effects.

Beta thalassemia major

Transfusion-dependent anemia from absent beta-globin production; chronic transfusions suppress ineffective erythropoiesis but require iron chelation, monitoring ferritin and organ iron, plus splenectomy only for specific indications.

Fanconi anemia

Inherited bone marrow failure with progressive pancytopenia, congenital anomalies, and cancer predisposition; diagnosis uses chromosome breakage testing, and care includes androgen, transplant, and cancer surveillance.

Diamond-Blackfan anemia

Pure red cell aplasia presenting in infancy with anemia, reticulocytopenia, and often congenital anomalies; corticosteroids are first-line, and transfusion with iron chelation supports nonresponders.

Hemophilia severity

Severity reflects factor activity: severe is below 1%, moderate 1–5%, and mild above 5%; severe disease causes spontaneous hemarthroses, while mild disease bleeds mainly after trauma or surgery.

von Willebrand disease

Mucocutaneous bleeding from quantitative or qualitative von Willebrand factor defects; desmopressin helps type 1, while factor concentrate is needed for severe or type 3 disease.

Immune thrombocytopenia

Isolated thrombocytopenia from platelet autoantibodies, diagnosed after excluding other causes; treat with IVIG or corticosteroids when bleeding or platelets are very low, not routinely by count alone.

DIC laboratory pattern

Disseminated intravascular coagulation shows prolonged PT and aPTT, low fibrinogen, elevated D-dimer, and thrombocytopenia; management centers on treating the trigger, with blood products for active bleeding.

Vitamin K deficiency bleeding

Neonatal bleeding due to insufficient vitamin K, prevented by intramuscular vitamin K at birth; treatment uses vitamin K and fresh frozen plasma or prothrombin complex for severe hemorrhage.

Antiphospholipid syndrome

Thrombosis or pregnancy morbidity with persistently positive antiphospholipid antibodies; anticoagulation is indicated for thrombosis, and duration depends on provoking factors and recurrence risk.

Acute lymphoblastic leukemia

Most common childhood cancer, arising from lymphoid precursors; treatment includes induction, consolidation, maintenance, and central nervous system prophylaxis, with risk and minimal residual disease guiding intensity.

Acute promyelocytic leukemia

AML subtype with t(15;17) and PML::RARA; start all-trans retinoic acid immediately when suspected because early coagulopathy and hemorrhage are major causes of death.

Burkitt lymphoma

Highly proliferative B-cell lymphoma with MYC translocation; intensive chemotherapy with central nervous system prophylaxis is required, and tumor lysis syndrome prevention is critical.

Neuroblastoma

Sympathetic nervous system tumor of childhood with elevated urine catecholamines and MIBG avidity; MYCN amplification and age/stage determine risk and treatment intensity.

Wilms tumor

Childhood renal tumor often presenting as an abdominal mass; treatment combines nephrectomy and chemotherapy, with radiation for higher stages or unfavorable features.

Retinoblastoma

Retinal tumor presenting with leukocoria or strabismus; germline RB1 mutations increase bilateral and secondary tumor risk, and therapy preserves vision when possible.

Tumor lysis syndrome

Oncologic emergency with hyperkalemia, hyperphosphatemia, hypocalcemia, and hyperuricemia; use aggressive hydration and rasburicase for high risk, avoiding rasburicase in G6PD deficiency.

Packed red cell dose

In children, 10–15 mL/kg of packed red cells typically raises hemoglobin by about 2 g/dL; transfuse for symptomatic anemia or defined thresholds, not a single universal hemoglobin value.

Irradiated blood products

Irradiation prevents transfusion-associated graft-versus-host disease by inactivating donor lymphocytes; indicated for hematopoietic stem cell transplant recipients, severe immunodeficiency, and selected intrauterine or neonatal transfusions.

Transfusion reaction first step

Stop the transfusion, maintain intravenous access, and assess for hemolysis, sepsis, overload, or allergic reaction; check patient and unit identification and notify the blood bank immediately.

Acute graft-versus-host disease

Donor lymphocytes attack recipient tissues, typically skin, gastrointestinal tract, and liver; prophylaxis uses immunosuppression, and treatment is high-dose corticosteroids with additional agents for steroid-refractory disease.

Hematopoietic engraftment

Neutrophil engraftment is commonly defined as an absolute neutrophil count above 500/µL for three consecutive days; platelet engraftment is sustained platelets above 20,000/µL without transfusion.

Autologous versus allogeneic HSCT

Autologous transplant uses the patient's own stem cells and has no graft-versus-tumor effect; allogeneic transplant uses donor cells and can produce graft-versus-tumor immunity but adds graft-versus-host disease risk.

Febrile neutropenia

Fever of 38.3°C once or 38.0°C sustained with severe neutropenia; obtain cultures and give empiric anti-pseudomonal antibiotics promptly, usually within 60 minutes.

Pneumocystis prophylaxis

Trimethoprim-sulfamethoxazole is standard prophylaxis during intensive chemotherapy or immunosuppression; alternative agents are used for sulfa allergy, and prophylaxis continues until immune recovery.

Anthracycline cardiotoxicity

Cumulative anthracycline exposure causes dose-dependent cardiomyopathy; monitor cardiac function by echocardiography, limit cumulative dose, and counsel survivors about later heart failure risk.

Fertility preservation counseling

Gonadotoxic therapy can impair future fertility; offer sperm banking, oocyte or embryo cryopreservation, and ovarian or testicular tissue options before treatment when time and age permit.

Survivorship care plan

A summary of cancer treatment and risks for late effects, including secondary cancers, endocrine, cardiac, and neurocognitive problems; it guides surveillance and transition to adult-focused care.

Pediatric assent

Children should receive developmentally appropriate information and provide assent when capable, while parents or guardians give legal consent; dissent is considered seriously in nonbeneficial research.