Heme/oncology Flashcards
What diagnosis should you consider in a teenager with leg pain who is going through a growth spurt?
Osteogenic sarcoma
What population is most likely to be diagnosed with osteogenic sarcoma? How do they present ?
Black teenager who presents with bone pain
What is the treatment of osteogenic sarcoma ? (2)
Amputation or limb salvage
Chemotherapy
What is the most common site of metastasis from osteogenic sarcoma?
Lung
What type of bone sarcoma presents with a soft tissue component?
Ewing sarcoma
What is the most common type of solid tumor in children?
Brain tumors
What diagnosis presents with left leg pain that is worse at night but relieved with ibuprofen?
Osteoid osteoma
What is the diagnosis of a femur with central radiolucent area surrounded by thick sclerotic bone?
Osteoid osteoma
What are 3 poor prognostic signs of ALL?
Age <2
WBC > 50
T cell precursor
Which sanctuary sites of ALL are most resistant to therapy and common sites of relapse?
CNS and testes
What is the correct first step in diagnosis in a child with unexplained lymphadenopathy and cough?
Chest X-ray
What diagnosis should you consider in a teenager who presents with non tender cervical lymph node and high WBCs?
Hodgkin lymphoma
What cells would you expect to find on lymph node biopsy in a patient with Hodgkin lymphoma ?
Reed sternberg
What is the first course of action in a patient with fever who has had splenectomy?
Sepsis workup
What is the difference between lymphoblastic and nonlymphoblastic non Hodgkin lymphoma?
Lymphoblastic is t. Cell origin and non lymphoblastic is b. Cell origin
Where are the 3 most common sites of mass secondary to non Hodgkin lymphoma ?
Head, neck, abdomen
What 3 tumors should you consider in a child with non tender abdominal mass?
Wilms
Neuroblastoma
Non Hodgkin lymphoma
What is the difference in bone marrow findings between leukemia and lymphoma?
Leukemia has >25% blasts
Lymphomas has <25% blasts
How is langerhans cell histiocytosis diagnosed?
Skin biopsy and electron microscopy
How is langerhans cell histiocytosis treated ? (3)
Surgery, steroids or chemo
What diagnosis should you consider in a patient with a lytic skull lesion and seborrheic rash?
Langerhans cell histiocytosis
What urinary problems are associated with langerhans cell histiocytosis?
Diabetes insipidus and excessive urination
Where is the most common place for presentation of neuroblastoma?
Adrenal gland
What is the prognosis of neuroblastoma?
Survival 95% if occurs prior to 1 year - poor prognosis of older than 1
How is neuroblastoma diagnosed?
Tumor biopsy or high urine VMA/HMA plus bone marrow tumor
What is the best imaging study to find neuroblastoma?
CT with contrast
What are 3 common lab abnormalities associated with neuroblastoma ?
Pancytopenia
Elevated ferritin
Elevated LDH
What is the most Likely cause of HTN in a patient with neuroblastoma?
Renal artery compression
What diagnosis should you consider in a patient with non tender abdominal mass, HTN and diarrhea ?
Neuroblastoma
What syndrome can occur from mediastinal tumor in a patient with neuroblastoma ?
Horner syndrome (compression of recurrent laryngeal nerve)
What is the chance that a woman with retinoblastoma in both eyes will have a child with retinoblastoma?
50% chance of any of her children having retinoblastoma
What is the chance that a woman with retinoblastoma in one eye will have a child with retinoblastoma?
5% chance
What are the 3 treatment options for retinoblastoma?
Surgery, chemotherapy or radiation
A child with retinoblastoma is at greater risk for later development of what Cancer?
Osteosarcoma
What is the appropriate management in a patient with vomiting who has a full fontanelle on exam and develops posturing after being given IV fluids?
Steroids &
Measures to reduce intracranial pressure
A child presents with grape-like mass protruding from the vagina, what is the likely diagnosis ?
Rhabdomyosarcoma
What are two types of cancer that can get worse or present after a traumatic injury ?
Rhabdomyosarcoma and osteogenic sarcoma
A child presents with constipation and on rectal exam you palpate a mass, what Cancer should you consider ?
Rhabdomyosarcoma
What diagnosis should you consider in a patient with non tender abdominal mass, HTN and gross hematuria?
Wilms tumor
What ophthalmologic finding is associated with Wilms tumor?
Aniridia
What two diagnoses are associated with hemihypertrophy?
Wilms tumor
Beckwith wiedemann
What 3 lab abnormalities should be monitored for in a patient getting initiation of chemotherapy ?
Hyperphosphatemia
Hyperkalemia
Hyperuricemia
What is the treatment for tumor lysis? (3)
Hydration
Alkalinization
Allopurinol
What should be done in a patient on chemotherapy who is exposed to varicella ?
Varicella zoster IG
What 4 things cause symptoms associated with anterior mediastinal masses?
Thymoma
Teratoma
Thyroid
T cell lymphoma
When should you intubate a patient suspected of having anterior mediastinal mass?
Never - airway compression Is below the vocal cords and it is dangerous to anesthetize these patients !!!
What diagnosis should you consider in a patient with a red face that is swollen as well as arm swelling and distended neck veins?
Superior vena cava syndrome
What is a potential side effect with use of cyclophosphamide? What can be done to prevent this side effect?
Hemorrhagic Cystitis - aggressive hydration and serial urinalysis
What is the potential side effect with use of bleomycin ?
Pulmonary fibrosis
What is a potential side effect with use of anthracycline (doxorubicin)?
Cardiac toxicity
What is a potential side effect of vincristine?
Neurotoxicity and SIADH
What side effect is associated with use of asparaginase?
Pancreatitis
What side effect is associated with use of procarbazine?
CNS toxicity
What hormone should be monitored in children having cranial radiation therapy?
Growth hormone
What potential side effect is associated with use of methotrexate?
Oral and GI ulcers
What is the cause of physiologic anemia of infancy?
Low erythropoietin production
What is the normal MCV for babies and toddlers?
70-90
What are 4 major causes of microcytic anemia in children ?
Iron deficiency
Thalassemia
Anemia of chronic illness
Lead poisoning
What diagnosis should you consider in a small for age Greek boy with anemia and large spleen?
Thalassemia major
What X-ray findings are compatible with thalassemia and why?
Thickened bone on X-ray
Skull with “hair on end” appearance - secondary to extramedullary hematopoiesis
What hemoglobin electrophoresis results are consistent with a diagnosis of beta thalassemia?
Low hemoglobin A1
High hemoglobin A2 (alpha delta)
High hemoglobin F
What are 2 long term complications of beta thalassemia and chronic transfusion therapy?
Cholelithiasis
Hemosiderosis
What are the results of hemoglobin electrophoresis in a patient with sickle cell anemia?
High hemoglobin F
High hemoglobin S
How long should iron deficiency anemia by treated?
Until 2 months after hgb levels are normal
What is the underlying cause of anemia in an 18 month old child with pallor whose diet consists mainly of milk?
Nutritional - low amount of iron is absorbed from cows milk
When is IV iron the correct treatment for anemia?
Only if child is Unable to take PO
What’s the difference in the RDW when comparing iron deficiency anemia and thalassemia?
RDW is high in iron deficiency
RDW is low in thalassemia
What are the ferritin and TIBC in anemia of chronic disease ?
Ferritin is high, TIBC is low
A patient presents with microcytic anemia and has a history of sickle cell disease …what is the likely cause of the anemia?
Iron deficiency
What is the appropriate treatment for anemia of chronic disease ?
Treatment of the underlying disease
Remember that patient will already have adequate iron stores
What is the gold standard for diagnosis of lead poisoning?
Whole blood lead level
What is this treatment for lead poisoning?
Chelation - penicillamine, dimercaprol and EDTA
When is the FEP (free erythrocyte protoporphyrin) elevated?
In lead poisoning and iron deficiency
What is the classic red blood cell appearance in lead poisoning?
Ringed sideroblasts
What type of anemia is caused by drinking goats milk?
Macrocytic anemia secondary to folate deficiency
What is the test that determines B12 absorption ?
Schilling test
What are 4 causes of B12 deficiency ?
Intrinsic factor
Bacterial overgrowth
Bowel resection
Infant of vegetarian mother
What diagnosis should you consider in a child with weakness, paresthesias and sore tongue whose lab findings reveal anemia?
Pernicious anemia
What is the cause of pernicious anemia?
Lack of intrinsic factor leads to impaired b12 absorption
What is the treatment of pernicious anemia?
IM cobalamin (aka B12)
You are presented with a patient who you suspect has folate deficiency, what is the most important step in diagnosis of the patient ?
Erythrocyte folic acid concentration is preferred over serum folate levels
*b12 deficiency must be ruled out before treatment!
What diagnosis should you suspect with red blood cells have “Heinz bodies”?
G6PD deficiency
What diagnosis should you consider in a black child with dark urine, jaundice and anemia shortly after treatment with Bactrim?
G6PD deficiency
What are the 2 products of red cell breakdown that are found to be elevated during hemolysis?
What level will be low during hemolysis?
Hemosiderin and bilirubin in urine
Low serum haptoglobin
When should G6PD testing be performed and why?
Several weeks after an acute episode because testing during an episode will be false negative
What is the difference between type A and type B G6PD deficiency?
Type A affects Africans and hemolysis occurs only after exposure to oxidants
Type B affects mediterraneans and has chronic hemolysis
What is the etiology (enzyme deficiency) of hereditary spherocytosis?
Spectrin deficiency
What diagnosis should you consider in a patient who presents with mild anemia, splenomegaly and intermittent jaundice whose MCHC is elevated ?
Hereditary spherocytosis
Why does hereditary spherocytosis cause hemolysis?
Defective red cell surface with fragile cells that break easily
What is the diagnostic method for hereditary spherocytosis?
Osmotic fragility test
What is the inheritance pattern of hereditary spherocytosis?
Autosomal dominant
What is the treatment (3) for hereditary spherocytosis?
Folic acid
Transfusion PRN
Splenectomy
What is the most common cause of aplastic crisis in children with hereditary spherocytosis?
Parvovirus
What amino acid substitution occurs in sickle cell anemia ?
Valine in place of glutamic acid at amino acid 6 of the beta globin
What vaccine is administered early in patients with sickle cell anemia?
Pneumococcal vaccine at age 2
What type of crisis should you consider in a sickle cell patient who presents with signs of shock?
Sequestration crisis due to pooling of blood in liver and spleen
What diagnosis should you consider in a patient with Howell Jolly bodies?
Sickle cell anemia
What should you consider in a 7 y/o patient with sickle cell anemia who presents with palpable spleen?
Hemoglobin SC or
Sickle cell plus thalassemia
You are presented with a sickle cell patient with chest pain, low O2 sats and infiltrate on chest X-ray, what’s the next best test?
ABG
What is the treatment for acute chest syndrome ?
Transfusion of pRBC or exchange transfusion
What is the best treatment for an African American child with symptoms of stroke?
Exchange transfusion followed by MRI
What imaging study should you obtain in a sickle cell patient with RUQ pain? Why?
Abdominal US to rule out gallstones which are associated with Sickle cell
How can u differentiate aplastic anemia from acute leukemia?
Aplastic anemia would have normal liver, no lymphadenopathy and normal LDH / uric acid
What side effect can occur w use of chloramphenicol ?
Aplastic anemia
A patient presents with renal abnormalities, abnormal skin pigmentation and macrocytic anemia, what diagnosis should you consider?
Fanconi anemia
What orthopedic problems are common in children with fanconi anemia?
Growth retardation
Absent/hypoplastic thumb
What future Cancer risk is likely in patients with fanconi anemia?
AML or myelodysplastic syndrome
What is the cure for aplastic anemia?
Bone marrow transplant
What are the two profound red cell anemias present in childhood? How do you differentiate the two?
Diamond Blackman anemia - presents in young infants and is chronic
Transient erythroblastopenia of childhood - presents in toddlers and self resolves
What type of anemia is due to an arrest in maturation of red blood cells?
Diamond blackfan
What anemia is due to suppression of erythroid production secondary to viral illness?
Transient erythroblastopenia of childhood
What diagnosis should you consider in a 2 month old with Abnormal facial features and severe anemia?
Diamond blackfan
Which type of severe childhood anemia is treated with steroids ?
Diamond blackfan
What type of reaction occurs when blood is not properly cross matched?
Hemolytic reaction
What type of transfusion reaction causes urticaria that typically resolves after antihistamine use?
Allergic reaction
How is risk for febrile nonhemolytic transfusion reaction reduced?
Use of leukocyte filtered blood
What diagnosis presents with WBCs full of lysosomal granules and abnormal chemotaxis?
Chediak Higashi
What diagnosis presents with frequent bad skin and lung infections in a child with fair skin, blond hair and blue eyes?
Chediak higashi
What are 3 common pathogens in patients with chediak Higashi?
Staph aureus
Strep pyogenes
Pneumococcus
How is chronic granulomatous disease diagnosed?
Nitroblue tetrazolium test measures oxidative burst
What types of infections are common in children with chronic granulomatous disease?
Recurrent bacterial (staph and gram negative) and fungal infections
What are 3 possible treatment options for chronic granulomatous disease?
Infection control
Interferon
Bone marrow transplant
What diagnosis presents with impaired wound healing, severe periodontal disease and a high WBC count in the absence of infection?
Leukocyte adhesion deficiency
What types of infections are common in patients with neutropenia?
Gran negative ulcers of the mucosa
What are 5 congenital neutropenias?
Cyclic neutropenia Kostmann syndrome (severe congenital neutropenia) Benign neutropenia Shwachman diamond Chediak Higashi
What are the two most common causes of acquired neutropenia?
Infection and drugs (macrolides)
What is the inheritance pattern of cyclic neutropenia ?
Autosomal dominant
What diagnosis would you consider in an 8 y/o make with oral lesions that occur once a month associated with low WBC count that lasts approx 1 week?
Cyclic neutropenia
What is the typical pathogenic cause of oral lesions associated with cyclic neutropenia?
Clostridium perfringens
What disease presents as an incidental finding of low WBCs that are not associated with signs of infection and typically resolves by age 2?
Chronic benign neutropenia
What is the inheritance pattern of kostmann agranulocytosis ?
Autosomal recessive
What diagnosis presents as severe neutropenia that does NOT rise and fall in cycles?
Kostmann syndrome
What diagnosis presents as short stature, steatorrhea and recurrent skin and URI infections as well as syndactyly?
Shwachman Diamond
How do you distinguish cystic fibrosis from shwachman diamond syndrome (other than a sweat chloride test of course)?
Both have pancreatic insufficiency and frequent infections but …SDS also presents with pancytopenia
What platelet problems are caused by use of aspirin and ibuprofen ?
Causes abnormal platelet function. Platelet count will be normal.
What is the first line treatment for ITP?
IVIG
When should platelet transfusion by indicated in patients with ITP?
Platelets lower then 20,000 or active bleeding
What is the prognosis of ITP in younger and older children?
50% resolve within 3 months in younger children
Can become chronic problem in children older than 10y/o
What should you be concerned about in a patient with ITP who presents with persistent headache?
Intracranial hemorrhage
What should you do in a 2 y/o patient with multiple bruises on the shins but normal platelets ?
Nothing - this is normal and typically not a sign of abuse
What diagnosis should you consider in a patient with purpura with normal platelet count?
Henoch schonlein purpura (or other vasculites)
What is anaphylaxtoid purpura?
Henoch Schonlein purpura
What diagnosis presents as a male child with diaper rash, bruising and chronic ear infections ?
Wiskott Aldrich Syndrome
How do you distinguish wiskott Aldrich syndrome from ITP?
ITP has large platelets
WAS has small platelets
What is the treatment of wiskott Aldrich ?
Bone marrow transplant
What is the inheritance pattern of wiskott Aldrich?
X linked (only in boys)
What syndrome presents with a large hemangioma and thrombocytopenia caused by consumptive coagulapathy? What are these patients at risk for developing?
Kasabach Merritt Syndrome - risk for DIC
What workup should be done for a child with excessive bleeding after circumcision ?
Congenital factor deficiency
What lab value is elevated when there is deficiency of vitamin K?
PT is elevated
Which are the vitamin K dependent factors in the coagulation cascade?
2, 7, 9 and 10
What lab value is abnormal in patients with hemophilia ?
Prolonged PTT
What should be done in a patient with hemophilia who suffers head trauma but is clinically doing well with no symptoms ?
Replacement of factors is indicated as soon as possible
What is the inheritance pattern of hemophilia ?
X linked recessive
What diagnosis should you consider in a patient with low platelets, high WBC count and abnormal X-ray of the radius ?
TAR
What’s the similarities & the main difference between diamond blackfan anemia and fanconi anemia?
Both with thumb abnormalities and low hgb but only fanconi has low platelets
What type of hemophilia typically presents in females?
None - they are X linked and only in males
Which factor level is affected in patients with Von willebrand disease?
Factor 8
What’s the usual hematologic cause of bleeding into a joint?
Hemophilia
What is the dangerous complication of bleeding into the antecubital region after a blood draw in a patient with hemophilia ?
Nerve compression - this is a medical emergency
What diagnosis should you consider in a female with menorrhagia who lab testing reveals normal PT and prolonged PTT? What is the next appropriate test?
Von willebrand - order Von willebrand factor activity
What should be the next step in a male with prolonged PTT and low Von willebrand factor activity?
Order factor 8 and 9 levels to rule out hemophilia
What is the management of Von willebrand in the case of mild bleeding?
What if there is major bleeding?
Intranasal DDAVP (stimate) Factor 8 concentrate if major bleeding
What is the inheritance pattern of Von willebrand disease?
Autosomal dominant
What is the mechanism of action of aminocaproic acid (amicar) in patients with Von willebrand ?
Inhibits fibrinolytic to stop mucosal bleeding
What diagnosis should you consider in a septic patient with low platelets, prolonged thrombin time and low fibrinogen?
DIC
