Hematology / Oncology Flashcards
Low hemoglobin, low mean corpuscular volume (MCV), low iron, low transferrin saturation, low ferritin, high red cell distribution width (RDW), Mentzer index (MCV/RBCs) >13 and high total iron-binding capacity (TIBC)
Iron deficiency anemia
Low hemoglobin, low iron, low/normal TIBC, normal/high ferritin level
Anemia of chronic disease
Mild anemia, low MCV, normal iron, normal TIBC, normal ferritin, normal RDW, Mentzer index
Thalassemia trait
Mild anemia, low MCV, normal iron, normal TIBC, normal ferritin, normal RDW, Mentzer index
Alpha thalassemia trait
A 12-month-old boy adopted from China with delayed growth, hepatosplenomegaly, jaundice, and “chipmunk facies”
Beta thalassemia major. (Alpha thalassemia major leads to severe anemia and hydrops fetalis in utero, typically incompatible with life without treatment)
Electrophoresis result showed: Hb A >98% with a small amount of Hb A2 visible
Normal electrophoresis
Electrophoresis of a 3-year-old child, result showed: Hb A is decreased to 94%, Hb A2 is increased at 5%, and Hb F is 1%
Beta thalassemia minor
After birth, hemoglobin electrophoresis result showed: No Hb A, Hb A2 of 4%, Hb F of 96%. No other abnormal hemoglobins seen
Beta thalassemia major
A 2-month-old premature infant has a Hgb 9.0 with normal MCV
Anemia of prematurity
How much will 10mL/kg of packed RBCs raise the hemoglobin?
~2g/dL
Excessive cow milk consumption (>16oz/day) and microcytic anemia
Iron deficiency anemia
What are the best initial laboratory tests in cases with suspected iron deficiency anemia?
CBC and reticulocyte count
What is the best indicator of response to iron therapy?
An increase in hemoglobin, reticulocyte count, and MCV within 1–4weeks
How long should iron therapy continue in cases of iron deficiency anemia?
At least 1–2months after anemia has been corrected to replete iron stores
What is the classic dose of iron in cases of iron deficiency anemia?
3–6mg/kg/day of “elemental iron”
A 2-year-old infant with a hemoglobin of 4g/dL, normal MCV, low reticulocyte count, normal ADA (adenosine deaminase activity), negative direct Coombs test and no signs of hemolysis
Transient erythroblastopenia of childhood
A 7-year-old child presents with pancytopenia, on exam also noted to have hypoplastic thumb and radius, hyperpigmentation, and abnormal facies
Fanconi anemia
A 4-month-old infant with severe anemia, high MCV (macrocytic), elevated ADA, and exam shows triphalangeal thumb
Diamond–Blackfan anemia
Macrocytic anemia, neutropenia, thrombocytopenia, exocrine pancreatic insufficiency, ring sideroblasts in the bone marrow
Pearson marrow-pancreas syndrome
Short stature, imperforate anus, hypoplastic teeth, frequent infections, macrocytic anemia, neutropenia, thrombocytopenia, and exocrine pancreatic insufficiency
Shwachman–Diamond syndrome
Child who consumes goat’s milk and has macrocytic anemia
Folic acid deficiency
Child whose family is strictly vegan and has macrocytic anemia
Suspect B12 deficiency. Supplement with B12
Child with macrocytic anemia, glossitis, abdominal pain, gait instability with positive anti-IF antibodies
Pernicious anemia (B12 deficiency due to IF antibodies)
Child with pallor, increased jaundice, splenomegaly, reticulocytosis, and normocytic hemolytic anemia. Peripheral smear shows RBCs without central pallor
Hereditary spherocytosis
An African-American boy recently started on Bactrim for UTI with sudden onset of dark urine, jaundice, and pallor. Splenomegaly on the exam. Labs are notable for anemia, reticulocytosis, indirect hyperbilirubinemia, low haptoglobin, and normal G6PD enzyme activity (during the episode). Peripheral smear is positive for Heinz bodies , blister , bite , occasional spherocyte
G6PD deficiency. Enzyme activity test is usually normal (false negative) during active hemolysis due to the destruction of older erythrocytes (that are G6PD deficient) and presence of younger erythrocytes and reticulocytes (that have normal/near-normal enzyme activity). The test should be repeated during remission, not during active hemolysis
Fava beans, primaquine, sulfa drugs, and nitrofurantoin are known to exacerbate which condition?
G6PD deficiency
Sickle cell anemia, swollen hands and feet, severe pain in hands and feet
Dactylitis
The most common cause of sepsis in patients with sickle cell disease
Streptococcus pneumoniae
Child with sickle cell disease presents with severe anemia, reticulocytosis, thrombocytopenia, and rapidly enlarging spleen
Splenic sequestration Next best step → transfusion of packed RBCs (monitor hemoglobin, expect additional rise in Hgb from auto-transfusion from spleen)
Child with sickle cell disease, fever, malaise, rash, severe anemia, and reticulocytopenia
Aplastic crisis → treatment packed RBCs transfusion as needed
Which virus is the most common cause of aplastic crisis?
Parvovirus B19
Common causes of morbidity and mortality in children with sickle cell disease
Infection, acute chest syndrome, stroke
Child with sickle cell anemia presents with fever, chest pain, tachypnea, shortness of breath, and new pulmonary infiltrate on imaging. Management?
Acute chest syndrome—start ceftriaxone + macrolide (to cover for atypical organisms). Avoid overhydration
Sickle cell patients are at higher risk of which type of organisms?
Encapsulated organisms—due to functional asplenia. Make sure vaccines are up to date
What is the most common reason for hospitalization in the child with sickle cell anemia? Management?
Vaso-occlusive pain crisis Treatment: IV hydration, NSAIDs, and opioids
Adolescent male with a painful erection that has lasted for several hours. Management?
Prolonged priapism—needs emergent evaluation and treatment. Ask patient to come to the ER, aspiration +/− irrigation, phenylephrine, pain control, possible surgical management
What is the most common cause of osteomyelitis in a child with sickle cell disease?
Salmonella
What is the next best step in a child with sickle cell disease and suspected osteomyelitis?
Imaging studies (MRI), blood culture, antibiotics (cover Salmonella and other Gram- negative bacilli, as well as S. aureus), consider biopsy for culture
Adolescent with sudden onset of fatigue, pallor, scleral icterus, and tachycardia, high reticulocyte count, positive direct antibody test. What is the most likely diagnosis?
Autoimmune hemolytic anemia (AIHA)
What is the next best step in the previous lifethreatening case of autoimmune hemolytic anemia (AIHA)?
Start steroids. Supportive care may include transfusion of the least incompatible packed RBC unit(s) Fever and absolute neutrophil count (ANC)
Fever and absolute neutrophil count (ANC)
Admit to the hospital, blood culture, IV antibiotics
Neutropenia for 1week every 3weeks, associated with gingivitis, pharyngitis, skin infections during nadir
Cyclic neutropenia
How to establish the diagnosis of cyclic neutropenia?
CBC 2–3 times per week for 6–8weeks
What is the best management of cyclic neutropenia?
Prophylactic granulocyte-colony stimulating factor (G-CSF). Immediate attention with fevers
Severe neutropenia from birth, oral ulcers, gingivitis, recurrent infections, ANC is low all the time
Kostmann syndrome
Persistent neck lymphadenopathy more than 1cm, fever, weight loss, night sweats, lack of response to oral antibiotics
Referral to a pediatric oncologist (lymph node biopsy)
Child with a supraclavicular lymph node for 2weeks. No other symptoms
Referral to a pediatric oncologist (must be biopsied or investigated)
Most common malignancy in infants
Neuroblastoma
Most common malignancy in childhood
Acute lymphocytic leukemia
Most common CNS tumor in children
Astrocytoma
Most common benign tumor of the liver in children
Infantile hemangioendothelioma (most commonly occurs in the first 6months of life, rarely seen in children >3years of age)
Toddler with an abdominal mass, ecchymosis, raccoon eye, myoclonic jerking, and random eye movements. The abdominal US is positive for a large suprarenal mass. Urine catecholamines are elevated (HVA and VMA )
Neuroblastoma
Child presents with gingivitis, hepatosplenomegaly, orbital chloromas, WBC >100,000. Peripheral smear shows Auer rods in blasts
Acute myelogenous leukemia
Chronic myelogenous leukemia is associated with which chromosome translocation?
Philadelphia chromosome t(9:22)
A 1-year-old with very large spleen, moderate leukocytosis (increased monocytes), xanthoma, eczema, and café-au-lait spots
Juvenile myelomonocytic leukemia (JMML). JMML has an association with NF1 and Noonan syndrome
Child with an abdominal mass presents with abdominal pain, weakness, lethargy, oliguria, edema, elevated lactate dehydrogenase (LDH) and uric acid, hyperkalemia, elevated phosphate, and low calcium
Burkitt lymphoma (tumor lysis syndrome)
What is the next best step in the previous case of tumor lysis syndrome?
Transfer immediately to oncology unit or PICU for supportive care, including hydration, correction of electrolytes—hyperkalemia, hyperphosphatemia, hyperuricemia, renal dysfunction. May even require hemodialysis
Microscopic picture of Hodgkin lymphoma
Reed-Sternberg cell
The most common type of lymphoma in children
Non-Hodgkin lymphoma
Most common malignant tumor of the kidney in children
Wilms tumor
Child with macroglossia and Wilms tumor
Beckwith–Wiedemann syndrome
Most common soft-tissue tumor in children
Rhabdomyosarcoma
Long-term complications of radiotherapy
Growth retardation, hypothyroidism, early onset coronary artery disease, pulmonary fibrosis, secondary malignancy
Complication of doxorubicin therapy
Cardiomyopathy
Complication of vincristine therapy
Neuropathy
This antineoplastic drug can cause renal impairment and ototoxicity
Cisplatin
Complication of methotrexate therapy
Mucositis , Renal and liver toxicity
Complication of cyclophosphamide therapy
Hemorrhagic cystitis
Common electrolyte abnormalities in tumor lysis syndrome?
Hyperkalemia, hyperuricemia, hyperphosphatemia, and hypocalcemia
A 12-year-old boy with pain and swelling above the knee, pain is worse at night; radiograph shows a bone lesion with Codman’s triangle and sunburst pattern
Osteosarcoma
A 16-year-old girl with back pain and limp, fever, weight loss, radiograph shows a mass on the iliac bone with a lytic lesion, onion-skin appearance
Ewing sarcoma
The translocation is commonly seen in patients with Ewing sarcoma
t (11;22)
Child with a painless, bony mass on the knee, radiograph shows broad base projection
Osteochondroma
Child with persistent pain in the lower part of the right femur, radiograph shows metaphyseal lucency surrounded by sclerotic bone, NSAIDs relieve the pain
Osteoid osteoma
Hemangioblastoma, pheochromocytoma, renal cell carcinoma, pancreatic cyst, and café-a- lait spots
Von–Hippel–Lindau disease
Impaired upward gaze, mid-dilated pupil, nystagmus, and lid retraction
Parinaud syndrome
New-onset head tilt or torticollis, early morning vomiting, headache, and gait disturbance
Posterior fossa brain tumor, e.g., medulloblastoma
Most common malignant CNS tumor in children
Medulloblastoma
Brain tumor with the best survival rate in children
Pilocytic astrocytoma
Child with recurrent headaches, growth failure, polydipsia, double vision
Craniopharyngioma
A 6-month-old child with strabismus, absent red reflex, and leukocoria
Retinoblastoma
The liver tumor that is associated with prematurity
Hepatoblastoma
The tumor that is associated with cryptorchidism
Hepatoblastoma
An infant with intracranial hemorrhage, prothrombin time (PT), partial thromboplastin time (PTT), and platelet count, fibrinogen and vWD panel is within normal limits
Factor XIII deficiency
Child with normal PT, very prolonged PTT, has no history of excessive bleeding even after injuries
Factor XII deficiency
A 5-year-old with upper respiratory tract infection 2weeks ago, presents with a bloody nose, petechial rash all over the body and oral mucosa. CBC is normal except platelet count is 12,000, peripheral smear shows very few large platelets
Idiopathic thrombocytopenic purpura (ITP)
What is the treatment in the previous case of ITP?
Observation if no signs of bleeding; if signs of bleeding, then treat with IVIG or steroids
A 2-year-old boy with recurrent infections, eczema, severe thrombocytopenia, and small platelets
Wiskott–Aldrich syndrome
Newborn with severe thrombocytopenia, maternal history of ITP or other autoimmune disorder
Neonatal ITP → give IVIG
Newborn with severe thrombocytopenia, maternal history of prior children with neonatal thrombocytopenia and no maternal history of autoimmunity
Neonatal alloimmune thrombocytopenia → transfuse maternal platelets (gold standard); however, difficult to obtain. The alternative option is donor platelets +/− IVIG, steroids
Unusual bleeding since birth, recurrent bruising, recurrent mucosal bleeding, low to normal platelet count, normal PT and PTT, normal fibrinogen, normal von Willebrand antigen and activity
Platelet function disorders, e.g., BernardSoulier syndrome, Glanzmann thrombasthenia
A 10-year-old with recurrent epistaxis, easy bruising, gingival bleeding, normal count and morphology of platelets, platelets agglutinate to ristocetin, poor platelet aggregation with adenosine diphosphate (ADP), epinephrine, and collagen
Glanzmann thrombasthenia (normal platelet count and size)
A 10-year-old with a suspected bleeding disorder, workup shows mild thrombocytopenia, with large abnormal platelets, platelets do not agglutinate to ristocetin but agglutinate to ADP, epinephrine, and collagen
Bernard–Soulier syndrome (large platelets, can have low platelet count)
Most appropriate management for life-threatening bleeding in a child with a known or suspected platelet function disorder
Infusion of platelets with normal function
A 48-h-old newborn presents with prolonged bleeding after circumcision, CBC shows severe thrombocytopenia. On exam no radii in both forearms but with normal thumbs
Thrombocytopenia with absent radii (TAR syndrome)
Male newborn with prolonged bleeding after circumcision, and prolonged PTT
Factor VIII or IX deficiency, or hemophilia A or B
A 15-year-old girl with excessive menstrual bleeding every month since menarche, normal PT and PTT, decrease in biological activity of ristocetin cofactor assay (rCoF)
Von Willebrand disease
A 4-year-old child with a recent history of vomiting and bloody diarrhea found to have thrombocytopenia, elevated BUN and creatinine, schistocytes on peripheral smear
Hemolytic uremic syndrome—occurs in infants and children after prodromal diarrhea, associated with bacteria particularly E. coli O157: H7, and Shigella dysenteriae
Child currently hospitalized in the PICU with prolonged PT, PTT, elevated D-dimer, thrombocytopenia, and decreased fibrinogen
Disseminated intravascular coagulation (DIC) Treatment: treat the underlying cause (e.g., antibiotics for sepsis). Supportive care with blood products—FFP +/− cryoprecipitate
A 17-year-old Caucasian boy with recurrent episodes of DVT along with a strong family history of DVT
Factor V Leiden mutation—resistance to activated protein C
A 2-year-old boy with oral ulcers, cradle cap-like rash, and gingivitis, with radiograph showing lytic lesions in the skull
Langerhans cell histiocytosis—Birbeck granules on electron microscopy
A 1-year-old sick-appearing child with fever, hepatosplenomegaly, pancytopenia, hypertriglyceridemia, and very elevated ferritin; presence of hemophagocytosis in bone marrow
Hemophagocytic lymphohistiocytosis (HLH)
Triad of iron deficiency anemia, esophageal webs, and dysphagia
Plummer-Vinson syndrome
