HEMATOLOGY / ONCOLOGY Flashcards by Verona Coralie Desulme

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

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Low hemoglobin, low iron, low/normal TIBC,
normal/high ferritin level

Anemia of chronic disease

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Mild anemia, low MCV, normal iron, normal TIBC, normal ferritin, normal RDW, Mentzer index < 13

Thalassemia trait

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Mild anemia, low MCV, normal iron, normal TIBC, normal ferritin, normal RDW, Mentzer index < 13, and normal electrophoresis (no elevated Hgb A2)

Alpha thalassemia trait

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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)

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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

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Electrophoresis result showed: Hb A > 98% with a small amount of Hb A2 visible

Normal electrophoresis

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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

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A 2-month-old premature infant has a Hgb 9.0 with normal MCV

Anemia of prematurity

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How much will 10 mL/kg of packed RBCs raise the hemoglobin?

~2 g/dL

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Excessive cow milk consumption (> 16 oz/day) and microcytic anemia

Iron deficiency anemia

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What are the best initial laboratory tests in cases with suspected iron deficiency anemia?

CBC and reticulocyte count

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What is the best indicator of response to iron
therapy?

An increase in hemoglobin, reticulocyte count,
and MCV within 1–4 weeks

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How long should iron therapy continue in cases of iron deficiency anemia?

At least 1–2 months after anemia has been
corrected to replete iron stores

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What is the classic dose of iron in cases of iron
deficiency anemia?

3–6 mg/kg/day of “elemental iron”

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A 2-year-old infant with a hemoglobin of 4 g/dL,
normal MCV, low reticulocyte count, normal ADA (adenosine deaminase activity), negative direct Coombs test and no signs of hemolysis

Transient erythroblastopenia of childhood

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A 7-year-old child presents with pancytopenia, on exam also noted to have hypoplastic thumb and radius, hyperpigmentation, and abnormal facies

Fanconi anemia

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A 4-month-old infant with severe anemia, high
MCV (macrocytic), elevated ADA, and exam shows triphalangeal thumb

Diamond–Blackfan anemia

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Macrocytic anemia, neutropenia,
thrombocytopenia, exocrine pancreatic
insufficiency, ring sideroblasts in the bone marrow

Pearson marrow-pancreas syndrome

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Short stature, imperforate anus, hypoplastic teeth, frequent infections, macrocytic anemia,
neutropenia, thrombocytopenia, and exocrine
pancreatic insufficiency

Shwachman–Diamond syndrome

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Child who consumes goat’s milk and has
macrocytic anemia

Folic acid deficiency

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Child whose family is strictly vegan and has
macrocytic anemia

Suspect B12 deficiency. Supplement with B12

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Child with macrocytic anemia, glossitis, abdominal pain, gait instability with positive anti-IF antibodies

Pernicious anemia (B12 deficiency due to IF
antibodies)

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Child with pallor, increased jaundice,
splenomegaly, reticulocytosis, and normocytic
hemolytic anemia. Peripheral smear shows RBCs without central pallor

Hereditary spherocytosis

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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

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) < 500. What is the next best step?

Admit to the hospital, blood culture, IV antibiotics

Neutropenia for 1 week every 3 weeks, 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 6–8 weeks

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 2 weeks. 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 6 months of life, rarely seen in children > 3 years 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 spo

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

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

Most common CNS tumor

Low-grade glioma (pilocytic astrocytoma is the most common LGG)

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

Gonadoblastoma

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 2 weeks 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., Bernard– Soulier 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)

HEMATOLOGY / ONCOLOGY Flashcards

(103 cards)