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