Heme/oncology Flashcards
1
Q
What diagnosis should you consider in a teenager with leg pain who is going through a growth spurt?
A
Osteogenic sarcoma
2
Q
What is the treatment of osteogenic sarcoma ? (2)
A
Amputation or limb salvage
Chemotherapy
3
Q
What is the most common site of metastasis from osteogenic sarcoma?
A
Lung
4
Q
What type of bone sarcoma presents with a soft tissue component?
A
Ewing sarcoma
5
Q
What is the most common type of solid tumor in children?
A
Brain tumors
6
Q
What diagnosis presents with left leg pain that is worse at night but relieved with ibuprofen?
A
Osteoid osteoma
7
Q
What is the diagnosis of a femur with central radiolucent area surrounded by thick sclerotic bone?
A
Osteoid osteoma
8
Q
What are 3 poor prognostic signs of ALL?
A
Age <2
WBC > 50
T cell precursor
9
Q
Which sanctuary sites of ALL are most resistant to therapy and common sites of relapse?
A
CNS and testes
10
Q
What is the correct first step in diagnosis in a child with unexplained lymphadenopathy and cough?
A
Chest X-ray
11
Q
What diagnosis should you consider in a teenager who presents with non tender cervical lymph node and high WBCs?
A
Hodgkin lymphoma
12
Q
What cells would you expect to find on lymph node biopsy in a patient with Hodgkin lymphoma ?
A
Reed sternberg
13
Q
What is the first course of action in a patient with fever who has had splenectomy?
A
Sepsis workup
14
Q
What is the difference between lymphoblastic and nonlymphoblastic non Hodgkin lymphoma?
A
Lymphoblastic is t. Cell origin and non lymphoblastic is b. Cell origin
15
Q
Where are the 3 most common sites of mass secondary to non Hodgkin lymphoma ?
A
Head, neck, abdomen
16
Q
What 3 tumors should you consider in a child with non tender abdominal mass?
A
Wilms
Neuroblastoma
Non Hodgkin lymphoma
17
Q
What is the difference in bone marrow findings between leukemia and lymphoma?
A
Leukemia has >25% blasts
Lymphomas has <25% blasts
18
Q
How is langerhans cell histiocytosis diagnosed?
A
Skin biopsy and electron microscopy
19
Q
How is langerhans cell histiocytosis treated ? (3)
A
Surgery, steroids or chemo
20
Q
What diagnosis should you consider in a patient with a lytic skull lesion and seborrheic rash?
A
Langerhans cell histiocytosis
21
Q
What urinary problems are associated with langerhans cell histiocytosis?
A
Diabetes insipidus and excessive urination
22
Q
Where is the most common place for presentation of neuroblastoma?
A
Adrenal gland
23
Q
What is the prognosis of neuroblastoma?
A
Survival 95% if occurs prior to 1 year - poor prognosis of older than 1
24
Q
How is neuroblastoma diagnosed?
A
Tumor biopsy or high urine VMA/HMA plus bone marrow tumor
25
What is the best imaging study to find neuroblastoma?
CT with contrast
26
What are 3 common lab abnormalities associated with neuroblastoma ?
Pancytopenia
Elevated ferritin
Elevated LDH
27
What is the most Likely cause of HTN in a patient with neuroblastoma?
Renal artery compression
28
What diagnosis should you consider in a patient with non tender abdominal mass, HTN and diarrhea ?
Neuroblastoma
29
What syndrome can occur from mediastinal tumor in a patient with neuroblastoma ?
Horner syndrome (compression of recurrent laryngeal nerve)
30
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
31
What is the chance that a woman with retinoblastoma in one eye will have a child with retinoblastoma?
5% chance
32
What are the 3 treatment options for retinoblastoma?
Surgery, chemotherapy or radiation
33
A child with retinoblastoma is at greater risk for later development of what Cancer?
Osteosarcoma
34
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
35
A child presents with grape-like mass protruding from the vagina, what is the likely diagnosis ?
Rhabdomyosarcoma
36
What are two types of cancer that can get worse or present after a traumatic injury ?
Rhabdomyosarcoma and osteogenic sarcoma
37
A child presents with constipation and on rectal exam you palpate a mass, what Cancer should you consider ?
Rhabdomyosarcoma
38
What diagnosis should you consider in a patient with non tender abdominal mass, HTN and gross hematuria?
Wilms tumor
39
What ophthalmologic finding is associated with Wilms tumor?
Aniridia
40
What two diagnoses are associated with hemihypertrophy?
Wilms tumor
| Beckwith wiedemann
41
What 3 lab abnormalities should be monitored for in a patient getting initiation of chemotherapy ?
Hyperphosphatemia
Hyperkalemia
Hyperuricemia
42
What is the treatment for tumor lysis? (3)
Hydration
Alkalinization
Allopurinol
43
What should be done in a patient on chemotherapy who is exposed to varicella ?
Varicella zoster IG
44
What 4 things cause symptoms associated with anterior mediastinal masses?
Thymoma
Teratoma
Thyroid
T cell lymphoma
45
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 !!!
46
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
47
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
48
What is the potential side effect with use of bleomycin ?
Pulmonary fibrosis
49
What is a potential side effect with use of anthracycline (doxorubicin)?
Cardiac toxicity
50
What is a potential side effect of vincristine?
Neurotoxicity and SIADH
51
What side effect is associated with use of asparaginase?
Pancreatitis
52
What side effect is associated with use of procarbazine?
CNS toxicity
53
What hormone should be monitored in children having cranial radiation therapy?
Growth hormone
54
What potential side effect is associated with use of methotrexate?
Oral and GI ulcers
55
What is the cause of physiologic anemia of infancy?
Low erythropoietin production
56
What is the normal MCV for babies and toddlers?
70-90
57
What are 4 major causes of microcytic anemia in children ?
Iron deficiency
Thalassemia
Anemia of chronic illness
Lead poisoning
58
What diagnosis should you consider in a small for age Greek boy with anemia and large spleen?
Thalassemia major
59
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
60
What hemoglobin electrophoresis results are consistent with a diagnosis of beta thalassemia?
Low hemoglobin A1
High hemoglobin A2 (alpha delta)
High hemoglobin F
61
What are 2 long term complications of beta thalassemia and chronic transfusion therapy?
Cholelithiasis
| Hemosiderosis
62
What are the results of hemoglobin electrophoresis in a patient with sickle cell anemia?
High hemoglobin F
| High hemoglobin S
63
How long should iron deficiency anemia by treated?
Until 2 months after hgb levels are normal
64
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
65
When is IV iron the correct treatment for anemia?
Only if child is Unable to take PO
66
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
67
What are the ferritin and TIBC in anemia of chronic disease ?
Ferritin is high, TIBC is low
68
A patient presents with microcytic anemia and has a history of sickle cell disease ...what is the likely cause of the anemia?
Iron deficiency
69
What is the appropriate treatment for anemia of chronic disease ?
Treatment of the underlying disease
| Remember that patient will already have adequate iron stores
70
What is the gold standard for diagnosis of lead poisoning?
Whole blood lead level
71
What is this treatment for lead poisoning?
Chelation - penicillamine, dimercaprol and EDTA
72
When is the FEP (free erythrocyte protoporphyrin) elevated?
In lead poisoning and iron deficiency
73
What is the classic red blood cell appearance in lead poisoning?
Ringed sideroblasts
74
What type of anemia is caused by drinking goats milk?
Macrocytic anemia secondary to folate deficiency
75
What is the test that determines B12 absorption ?
Schilling test
76
What are 4 causes of B12 deficiency ?
Intrinsic factor
Bacterial overgrowth
Bowel resection
Infant of vegetarian mother
77
What diagnosis should you consider in a child with weakness, paresthesias and sore tongue whose lab findings reveal anemia?
Pernicious anemia
78
What is the cause of pernicious anemia?
Lack of intrinsic factor leads to impaired b12 absorption
79
What is the treatment of pernicious anemia?
IM cobalamin (aka B12)
80
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!
81
What diagnosis should you suspect with red blood cells have "Heinz bodies"?
G6PD deficiency
82
What diagnosis should you consider in a black child with dark urine, jaundice and anemia shortly after treatment with Bactrim?
G6PD deficiency
83
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
84
When should G6PD testing be performed and why?
Several weeks after an acute episode because testing during an episode will be false negative
85
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
86
What is the etiology (enzyme deficiency) of hereditary spherocytosis?
Spectrin deficiency
87
What diagnosis should you consider in a patient who presents with mild anemia, splenomegaly and intermittent jaundice whose MCHC is elevated ?
Hereditary spherocytosis
88
Why does hereditary spherocytosis cause hemolysis?
Defective red cell surface with fragile cells that break easily
89
What is the diagnostic method for hereditary spherocytosis?
Osmotic fragility test
90
What is the inheritance pattern of hereditary spherocytosis?
Autosomal dominant
91
What is the treatment (3) for hereditary spherocytosis?
Folic acid
Transfusion PRN
Splenectomy
92
What is the most common cause of aplastic crisis in children with hereditary spherocytosis?
Parvovirus
93
What amino acid substitution occurs in sickle cell anemia ?
Valine in place of glutamic acid at amino acid 6 of the beta globin
94
What vaccine is administered early in patients with sickle cell anemia?
Pneumococcal vaccine at age 2
95
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
96
What diagnosis should you consider in a patient with Howell Jolly bodies?
Sickle cell anemia
97
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
98
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
99
What is the treatment for acute chest syndrome ?
Transfusion of pRBC or exchange transfusion
100
What is the best treatment for an African American child with symptoms of stroke?
Exchange transfusion followed by MRI
101
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
102
How can u differentiate aplastic anemia from acute leukemia?
Aplastic anemia would have normal liver, no lymphadenopathy and normal LDH / uric acid
103
What side effect can occur w use of chloramphenicol ?
Aplastic anemia
104
A patient presents with renal abnormalities, abnormal skin pigmentation and macrocytic anemia, what diagnosis should you consider?
Fanconi anemia
105
What orthopedic problems are common in children with fanconi anemia?
Growth retardation
| Absent/hypoplastic thumb
106
What future Cancer risk is likely in patients with fanconi anemia?
AML or myelodysplastic syndrome
107
What is the cure for aplastic anemia?
Bone marrow transplant
108
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
109
What type of anemia is due to an arrest in maturation of red blood cells?
Diamond blackfan
110
What anemia is due to suppression of erythroid production secondary to viral illness?
Transient erythroblastopenia of childhood
111
What diagnosis should you consider in a 2 month old with Abnormal facial features and severe anemia?
Diamond blackfan
112
Which type of severe childhood anemia is treated with steroids ?
Diamond blackfan
113
What type of reaction occurs when blood is not properly cross matched?
Hemolytic reaction
114
What type of transfusion reaction causes urticaria that typically resolves after antihistamine use?
Allergic reaction
115
How is risk for febrile nonhemolytic transfusion reaction reduced?
Use of leukocyte filtered blood
116
What diagnosis presents with WBCs full of lysosomal granules and abnormal chemotaxis?
Chediak Higashi
117
What diagnosis presents with frequent bad skin and lung infections in a child with fair skin, blond hair and blue eyes?
Chediak higashi
118
What are 3 common pathogens in patients with chediak Higashi?
Staph aureus
Strep pyogenes
Pneumococcus
119
How is chronic granulomatous disease diagnosed?
Nitroblue tetrazolium test measures oxidative burst
120
What types of infections are common in children with chronic granulomatous disease?
Recurrent bacterial (staph and gram negative) and fungal infections
121
What are 3 possible treatment options for chronic granulomatous disease?
Infection control
Interferon
Bone marrow transplant
122
What diagnosis presents with impaired wound healing, severe periodontal disease and a high WBC count in the absence of infection?
Leukocyte adhesion deficiency
123
What types of infections are common in patients with neutropenia?
Gran negative ulcers of the mucosa
124
What are 5 congenital neutropenias?
```
Cyclic neutropenia
Kostmann syndrome (severe congenital neutropenia)
Benign neutropenia
Shwachman diamond
Chediak Higashi
```
125
What are the two most common causes of acquired neutropenia?
Infection and drugs (macrolides)
126
What is the inheritance pattern of cyclic neutropenia ?
Autosomal dominant
127
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
128
What is the typical pathogenic cause of oral lesions associated with cyclic neutropenia?
Clostridium perfringens
129
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
130
What is the inheritance pattern of kostmann agranulocytosis ?
Autosomal recessive
131
What diagnosis presents as severe neutropenia that does NOT rise and fall in cycles?
Kostmann syndrome
132
What diagnosis presents as short stature, steatorrhea and recurrent skin and URI infections as well as syndactyly?
Shwachman Diamond
133
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
134
What platelet problems are caused by use of aspirin and ibuprofen ?
Causes abnormal platelet function. Platelet count will be normal.
135
What is the first line treatment for ITP?
IVIG
136
When should platelet transfusion by indicated in patients with ITP?
Platelets lower then 20,000 or active bleeding
137
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
138
What should you be concerned about in a patient with ITP who presents with persistent headache?
Intracranial hemorrhage
139
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
140
What diagnosis should you consider in a patient with purpura with normal platelet count?
Henoch schonlein purpura (or other vasculites)
141
What is anaphylaxtoid purpura?
Henoch Schonlein purpura
142
What diagnosis presents as a male child with diaper rash, bruising and chronic ear infections ?
Wiskott Aldrich Syndrome
143
How do you distinguish wiskott Aldrich syndrome from ITP?
ITP has large platelets
| WAS has small platelets
144
What is the treatment of wiskott Aldrich ?
Bone marrow transplant
145
What is the inheritance pattern of wiskott Aldrich?
X linked (only in boys)
146
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
147
What workup should be done for a child with excessive bleeding after circumcision ?
Congenital factor deficiency
148
What lab value is elevated when there is deficiency of vitamin K?
PT is elevated
149
Which are the vitamin K dependent factors in the coagulation cascade?
2, 7, 9 and 10
150
What lab value is abnormal in patients with hemophilia ?
Prolonged PTT
151
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
152
What is the inheritance pattern of hemophilia ?
X linked recessive
153
What diagnosis should you consider in a patient with low platelets, high WBC count and abnormal X-ray of the radius ?
TAR
154
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
155
What type of hemophilia typically presents in females?
None - they are X linked and only in males
156
Which factor level is affected in patients with Von willebrand disease?
Factor 8
157
What's the usual hematologic cause of bleeding into a joint?
Hemophilia
158
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
159
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
160
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
161
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
```
162
What is the inheritance pattern of Von willebrand disease?
Autosomal dominant
163
What is the mechanism of action of aminocaproic acid (amicar) in patients with Von willebrand ?
Inhibits fibrinolytic to stop mucosal bleeding
164
What diagnosis should you consider in a septic patient with low platelets, prolonged thrombin time and low fibrinogen?
DIC
165
What population is most likely to be diagnosed with osteogenic sarcoma? How do they present ?
Black teenager who presents with bone pain
