Blood and neoplastic disorders1


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Blood and neoplastic disorders1

  1. 1. Blood and Neoplastic Disorders<br /><ul><li>General Aspects
  2. 2. Recognize by Hx
  3. 3. Recognize recurrent bacterial infections as a manifestation of quantitative or qualitative leukocyte disorders
  4. 4. Know that children with severe neutropenia may become infected with their own skin and bowel flora
  5. 5. The most common offending organisms are staph aureus and gram-negative bacteria
  6. 6. Recognize by PE
  7. 7. Recognize palpable bruises in areas not exposed to trauma as distinctly abnormal
  8. 8. Recognize mucosal ulcerations as a sign of neutropenia
  9. 9. Mucosal ulcerations and also gingival inflammation
  10. 10. Distinguish between bruising due to TCP and normal bruising in an active child
  11. 11. Bruises seen in normal active children are usually pretibial; bruises seen on kids with TCP are often on the arms and trunk as well as the legs
  12. 12. Recognize that child abuse may be a cause of bruising in a child with a normal platelet count
  13. 13. Bruises related to abuse tend to be more generalized and do not have associated petechiae or TCP
  14. 14. Recognize that vasculitic disorders my be a cause of bruising or purpura in a child with a normal (or increased) platelet count
  15. 15. Know that TCP or functional plt disorders may cause bruising, petechiae, epistaxis, or GI bleeding but rarely cause deep muscle or joint bleeding
  16. 16. Deep muscle or joint bleeding are indicative of hemophlia and other blood coagulation disorders
  17. 17. Formulate the ddx for a pt with a purpuric rash
  18. 18. Recognize that children with hemihypertrophy and somatic overgrowth syndromes should be periodically evaluated for the development of embryonal tumors
  19. 19. This includes Beckwith-Wiedman
  20. 20. Patients with these syndromes have a high incidence of Wilms and hepatoblastoma
  21. 21. Surveillance for development of Wilms include following serum AFP levels, US for microscopic hematuria, and also abd u/s q3months until age 5years
  22. 22. WAGR syndrome: Wilms, aniridia, GU abnormalities, retardation)
  23. 23. Interpretation of lab results
  24. 24. Recognize the physiologic anemia of infancy and understand that further lab eval is not necessary
  25. 25. Nadir is reached at about age 2 months with avg hgbs of about 9-11
  26. 26. At birth there is a drop in epo production due to the dramatic increase in arterial PaO2
  27. 27. Fun fact: RBC lifespan during the first 6-8 weeks of life is 90 days, then increases to the usual 120 day lifespan after that age
  28. 28. Understand the concept of physiologic anemia
  29. 29. Know that Fe deficiency anemia and thalassemia minor are the most common causes of microcytic anemia
  30. 30. Know that a total leukocyte count and leukocyte differential count are needed to dx neutropenia
  31. 31. May have a normal WBC count but an ANC in the neutropenic range
  32. 32. Know that neutropenia is usually defined as a neutrophil count <1000
  33. 33. Mild neutropenia = ANC 1000-1500
  34. 34. Moderate neutropenia = ANC 500-1000
  35. 35. Severe neutropenia = ANC <500
  36. 36. Recognize that bleeding time is a test of platelet function and blood vessel function
  37. 37. Also know that we don’t do this test anymore
  38. 38. Recognize that TCP is defined as a plt count <150
  39. 39. Understand the normal variation in hgb concentration and MCV during childhood
  40. 40. MCV falls during the first 6-12 months to an approx nadir of ~77 and then rises through rest of childhood and adolescence
  41. 41. Hgb initially falls, as above
  42. 42. In the rest of childhood it keeps up with growth, gradually increasing; then in adolescence it further increases so that male > female hgb (due to androgen)
  43. 43. Erythrocyte disorders
  44. 44. Nutritional anemias
  45. 45. Understand that Fe deficiency anemia causes nonhematologic effects such as behavior and learning disturbances
  46. 46. Unclear whether these effects are reversible with Fe treatment; goal of Fe tx is to try and prevent them from occurring in the first place
  47. 47. These effects can occur even without presence of anemia; so if the hgb is normal but the MCV is low, need to tx with Fe
  48. 48. Know that dietary deficiency is the most common cause of Fe deficiency anemia in young children
  49. 49. Dietary iron is absorbed in the duodenum
  50. 50. The presence of acidic foods enhances the absorption of iron
  51. 51. Human milk has less iron than formuka, but it is mush more well absorbed
  52. 52. Heme iron (i.e. from meats and shellfish_ is better absorbed than non-heme iron (from grains, vegtables, etc)
  53. 53. Calcium inhibits Fe absorption
  54. 54. Know that cow milk contains very little bio-available iron and that an infant with Fe deficiency anemia often drinks large amount of cow milk
  55. 55. Very important to get a good dietary hx
  56. 56. Understand the need to look for bleeding as a cause of Fe deficiency anemia in a child with a normal diet
  57. 57. Look for melena, hematochezia, heavy menses; also check for fecal occult blood
  58. 58. Know how to dx Fe deficiency anemia
  59. 59. Microcytosis
  60. 60. Elevated RDW; decreased retic, RBC number and mean cellular hgb
  61. 61. More advanced tests: ferritin (total body iron stores) may be low, but in cases of acute illness it may be elevated as it is an acute phase reactant; TIBC may me elevated; transferring saturation is low
  62. 62. Mentzer index = MCV/RBC; if it is >13 it is indicative of Fe deficiency, if <13 it is indicative of a thalassemia (but this is only a screen)
  63. 63. Know that tx for Fe deficiency with Fe sulfate should continue after the hgb concentration has returned to normal
  64. 64. Should see increases in MCV, hgb and retic counts in about 1-4 weeks, and these should be checked in that timeframe to see if there is a result of the Fe tx
  65. 65. Usual dose is 6mg/kg/day of elemental Fe and it can be in divided doses. Giving it with vitamin C can help improve absorption
  66. 66. Reason for continuing the tx beyond the initial improvement is to replenish iron stores and prevent repeat problems
  67. 67. Know that IM injxns or erythrocyte infusions are not appropriate for a child with routine nutritional Fe deficiency
  68. 68. Recognize vit B12 or folate as a cause of macrocytic anemia
  69. 69. Macrocytosis = MCV >100
  70. 70. Megaloblastic = presence of macro-ovalocytes snd hypersegmented neutrophils
  71. 71. B12 associated sx
  72. 72. Glossitis (smooth painful tongue)
  73. 73. Neurologic sx including ataxia, paresthesias and difficulty walking as well as decreased reflexes, dimished vibratory and positon sense
  74. 74. Altered mental status in late stages
  75. 75. Document the dx of B12 or folate deficiency with specific measurement of serum B12 concentration or serum or erythrocyte folic acid concentrations before beginning replacement tx
  76. 76. Regarding folate, the RBC / erythrocyte folate level is the one to check because the serum folate level is variable and may not acutely reflect that folate status
  77. 77. Know that B12 deficiency may occur following small bowel resection or as a result of a maternal vegan diet in a child who is exclusively breastfed
  78. 78. If there are gut probs in the patient, think B12
  79. 79. Remember intrinsic factor (made in the parital cells) required to absorb the B12 in the ileum. Also requires transcobalamin II within the cells to be converted to active form
  80. 80. Congenital pernicious anemia = abnormal or deficient IF; usually declares itself in first year of life
  81. 81. Juvenile pernicious anemia= anti-IF autoantibodies; presents later than congenital
  82. 82. Also note that PPIs and metformin are two common meds that can lead to B12 deficiency
  83. 83. Know that ingestion of goat milk as a principal source of nutrition in infancy is a cause of folate deficiency
  84. 84. Hemolytic anemias
  85. 85. Know that G6PD is a common X-linked disorder
  86. 86. Hence males more affected than females
  87. 87. Know that sudden onset of pallor and anemia may be a manifestation of G6PD deficiency
  88. 88. Indicates an acute hemolytic process
  89. 89. Should also be considered in any newborn who manifests jaundice in the first 24 horus after borth, is jaundce depiste being exclusively formula fed, or has a fam hx of RBC disorders
  90. 90. Know that causes of hemolysis in pts with G6PD and manage appropriately
  91. 91. Main thing is to avoid agents of oxidative stress
  92. 92. Meds: sulfonamides, antimalarial drugs, nitrofurantoin, cipro
  93. 93. Also illnesses can increase the oxidative stress and led to more hemolysis
  94. 94. Tx = supportive care, giving PRBCs if needed
  95. 95. Be aware of the clinical differences in the forms of G6PD seen in patients with different ethnic backgrounds
  96. 96. More severe in those of Asian and Meditarranean descent, and they need to avoid the meds above, as well as ASA and APAP
  97. 97. Understand that sickle cell disease can be dxed at birth
  98. 98. Newborn screen
  99. 99. Know that kids with SCD are particularly vulnerable to death from overwhelming bacterial sepsis and require early evaluation and tx when febrile
  100. 100. Clinical guidelines for intervention:
  101. 101. All pts with SCD who develop fver >101 is mandated and administration os a 3rd generation cephalosporin (ceftriaxone, cefotaxime) plus clinda or vanco for severe infections is the current practice (Per PIR article, 2007)
  102. 102. The spleen is the first organ to experience major damage in SCD; therefore infection w encapsulated orgaisms (strep penumo) is especially concerning
  103. 103. Recognize the findings of an enlarged spleen and worsening anemia as indicative of sequestration crisis in SCD
  104. 104. Understand that immediate intervention with IVFs and/or blood is the tx for acute sequestration crises
  105. 105. Recognize painful swelling of the hands or feet as a manifestation of SCD in young children
  106. 106. Understand the use of ppx penicillin in kids with SCD
  107. 107. Due to loss of functional spleen and susceptibility to strep pneumo
  108. 108. Main rec is to start by age 3months and continue it up to age 5years
  109. 109. Growing resistance PCN and cephalosposins, hence tx as above
  110. 110. Recognize increasing pallor, decreased hgb concentration, and a decreased retic count as findings in an aplastic crisis in SCD
  111. 111. Know that acute chest syndrome and painful crises are common manifestations of SCD
  112. 112. Acute Chest = radiographic appearance of a new pulmonary infiltrate along with a fever
  113. 113. Can be due to infection, VOC, fat embolism
  114. 114. Tx = broad spectrum abx, including a cephalosporin and a macrolide, as well as oxygen, hydration, incentive spriometry and early intervention with simple transfusion tx for associated hypoxia or hct <18%
  115. 115. Painful crises are most common cause of hospitalization
  116. 116. Frequent admissions for pain crises (>6/year) are a known risk factor for early death in SCD
  117. 117. Management should include aggressive pain management with age appropriate patient controlled analgesia (morphine or hydromorphone), NSAIDs (ibup or ketorolac), hydration, PT, and ancillary therapies like guided therapy/relaxation, etc
  118. 118. Know that thalassemia major usually presents as a severe hypochromic, microcytic anemia with enlargement of the liver and/or spleen
  119. 119. Recognize the association of cholelithiasis in patients with SCD
  120. 120. 42% of patients with Hb-SS have cholelithiasis by dolescence.
  121. 121. Cholecystectomy is only indicated if they hav suffer abd pain or cholestatic jaundice due to common duct obstruction; if they get cholecystitis then they are txed with abx and supportive care and have elective cholcystectomy weeks after the acute event
  122. 122. Know that priapism os a common manifestsation of SCD
  123. 123. Understand the complications of erythrocyte infusion in a child with autoimmune hemolytic anemia
  124. 124. The transfused erythrocytes will also be destroyed
  125. 125. Know that ABO incompatibility may cause anemia in a first born child, but Rh incompatibility rarely does
  126. 126. Know that progressive and severe anemia may occur at 4-8 weeks of age in infants with severe Rh or ABO incompatibility
  127. 127. Aplastic and hypoplastic erythrocyte disorders
  128. 128. Distinguish between that clinical characteristics of Diamond Blackfan syndrome and transient erythroblastopenia of childhood
  129. 129. TEC: usually occurs around age 2; rarely in the first year after birth
  130. 130. PE findings normal other than pallor and other si/sx of anemia
  131. 131. Possible h/o recent viral illness
  132. 132. Other labs usually WNL
  133. 133. Tx with transfusion of hgb <5
  134. 134. DBA: often dxed in first year of life
  135. 135. Associated PE findings include abn facies, thumb abnormalities, congenital heart diseases
  136. 136. Lab with macrocytic anemia and reticulocytopenia
  137. 137. Have insensitivity to epo
  138. 138. Know the signs, symptoms and lab findings of transient erythroblastopenia of childhood
  139. 139. See above
  140. 140. Understand the role of erythrocyte transfusions in transient erythroblastopenia of childhood
  141. 141. Only for hgb <5; usually not needed if >5
  142. 142. Therapeutic Approaches
  143. 143. Understand the risk of transmitting infectious diseases during blood transfusions
  144. 144. HIV: about 1 in 2.1 million
  145. 145. HCV: about 1 in 1.9 million
  146. 146. HBV: about 1 in 200,000
  147. 147. Recognize that erythrocyte transfusions may be associated with hemolytic, febrile and urticarial reactions
  148. 148. Febrile nonhemolytic transfusion reaction (FNHTR)
  149. 149. Occur in about 0.2% of transfusions and are likely due to cytokine release from the donor blood WBCs; can be minimized with leukocyte reduced blood
  150. 150. Premed with APAP does not decrease its incidence
  151. 151. Urticarial reactions/Allergic reactions
  152. 152. About 0.4% of transfusions
  153. 153. If occurs need to give antihistamines and hold the transfusion; if the sx resolve quickly then the transfusion can likely be resumed
  154. 154. Premed with antihistamines does not reduce incidence
  155. 155. Kids with h/o allergic reactions may do better with plasma reduced PRBCs or washed RBCs
  156. 156. Hemolytic reactions: can be immediate or delayed
  157. 157. Immediate: due to high-titer Abs and usually due to ABO incompatible blood
  158. 158. Si/sx = fever, chills, N/V, abd or back pain, tachycardia and hypotension
  159. 159. Must immediately stop the transfusion and give supportive care for hemolysis and possibl DIC, shock, renal failure
  160. 160. Delayed: due to primary immunization or due to anamnestic response from a low level alloantibody
  161. 161. Primary usually happen about 2-3 weeks after transfusion with si/sx of hemolysis
  162. 162. Anamnestic usually happen with days and can have same si/sx as immediate reactions, although more mild but may still need supportive care
  163. 163. Understand the role of erythrocyte transfusions in the management of anemia
  164. 164. See examples in prior sections
  165. 165. Understand the role of erythropoietin in the management of anemia or renal failure and chronic inflammatory disease
  166. 166. This is all up for debate
  167. 167. It might be helpful for those with CKD who would have reduced levels of epo due to their kidney probs, but what the actual role and benefit can be is currently being questioned (my editorialization)
  168. 168. Leukocyte disorders
  169. 169. Quantitative leukocyte disorders
  170. 170. Recognize that congenital neutropenia may be persistent or cyclical, and manage appropriately
  171. 171. Cyclical neutropenia:
  172. 172. Usually cycles of about 21 days; often with fevers and mouth ulcers during the nadir of the WBC count
  173. 173. Neutropenia often lasts 3-4 days
  174. 174. Dx can be difficult b/c by the time they present they may have a nrmal or near normal WBC count; therefore if it suspected, they will need to have their counts checked 2-3 times a week for 4-6 weeks in order to determine if there is a cycle
  175. 175. Does not appear to be a risk factor for myelodysplasia or AML
  176. 176. During the nadir of the WBC count they may benefit from GCSF
  177. 177. Persistent: severe congenital neutropenia; multiple varieties
  178. 178. Presentation may be with umbilical infection, pyoderma, oral ulcers, pulmonary infections or perineal infections
  179. 179. ANC often less than 500
  180. 180. GCSF is used to support them
  181. 181. Have a higher risk of developing myelodyplastic syndromes and AML
  182. 182. Recognize neutropenia as a sign of overwhelming bacterial sepsis
  183. 183. Can be sign of overwhelming bacterial sepsis (basically consumptive)
  184. 184. Understand that neutropenia may be drug induced and, if so, should lead to d/c of the drug
  185. 185. Leading offenders include Anagelsics/NSAIDs, certain abx (chloramphenical, sulfa, pcn), anticonvulsants (phenytoin, carbamazepine), PTU, procainamide, quinidine, cimetidjne, ranitidine
  186. 186. Recognize that common viral infections may cause transient neutropenia that does not require specific tx
  187. 187. Consider a f/u CBC after the condition has resolved in order to see that it has improved
  188. 188. Qualitative leukocyte disorders
  189. 189. Recognize that a child with recurrent bacterial infections and a normal neutrophil count may have abnormal neutrophil function
  190. 190. Therapeutic approaches
  191. 191. Understand the role of growth factors in the tx of neutropenia
  192. 192. Platelet disorders
  193. 193. Quantitative platelet disorders
  194. 194. Know that TCP in a newborn infant may be a sign of bacterial sepsis and, in an ill child, should lead to appropriate culture and antibiotic therapy
  195. 195. Usually have TCP due to platelet destruction caused by vasculitis, endotoxin, immune complexes or DIC
  196. 196. Plt transfusions are recommended when the count is <10, whenthere is difficult to control bleeding or when an invasive procedure is planned
  197. 197. Recognize that a hx of medications should be part of the eval of a child with tcp
  198. 198. Valproic acid and other anticonvulsanrs are notable for causing TCP
  199. 199. Recognize that the presence of TCP in a newborn infant with microcepahly a=or other congenital abnormalities may be due to a congenital viral infection such as CMV
  200. 200. Recognize TCP, eczematoid rash and recurrent infections as signs of Wiskott-Aldrich syndrome
  201. 201. Know that a rapidly enlarging hemangioma should lead to a plt count to check for TCP
  202. 202. Manage the TCP associated with TAR syndrome
  203. 203. Maintain plts >15,000 with plt transfusions
  204. 204. The TCP is usually self-limited and resolves by age 2 years
  205. 205. Need to avoid trauma and elective surgeries until the TCP resolves
  206. 206. Genetics consults are recommended and need to address recurrence risks
  207. 207. Note: the TCP is not responsive to steroids or IVIg, it is actually due to a hyporegenerative marrow; don’t need to do a bone marrow bx
  208. 208. Know that ITP is characterized by a low plt count and normal or increased production of plts in the marrow
  209. 209. This is the most common cause of isolated TCP in otherwise well children
  210. 210. Bone marrow bx isn’t part of the work up, and should only be considered with referral to a hematologist
  211. 211. Understand that most children with ITP will recover in less than one year without tx
  212. 212. Tx is available, but do not alter the natural hx of acute ITP, neither preventing its progression to chronicity or accelerating disappearance of the offending antibody
  213. 213. Know that the common presenting sx of ITP is increased bruising
  214. 214. Usually a sudden onset of petechiae and bruising in a previously healthy child; may also have some mucosal bleeding
  215. 215. Know that corticosteroids and IVIg usually increase the plt count in children with ITP but do not alter the natural course (e.g. length of the dz)
  216. 216. Steroids help raise the count faster than no tx, and IVIg helps raise the count faster than steroids
  217. 217. No data show that drug treatment helps prevent serious bleeding, including ICH
  218. 218. Note, British panels recommend observation, American panels recommend intervention; overall more study is needed per the PIR article
  219. 219. Understand that ASA or other drugs that interfere with plt fxn should not be given to children w ITP or other qualitative or quantitative plt d/os
  220. 220. Recognize persistent or severe HA as a sign if ICH in ITP
  221. 221. Need to have imaging and get drug tx promptly
  222. 222. Know that splenectomy is not appropriate tx at the onset of ITP in a child who is not having major bleeding problems
  223. 223. 5-10% of patients will not respond to the splenectomy and there is no way to predict it
  224. 224. Usually viewed as a last resort
  225. 225. Know that a mother with ITP may have an infant with TCP and know how to manage the infant
  226. 226. Usually these are “well” infants, they rarely have bleeding probs
  227. 227. If they do have bleeding issues then IVIg is the tx of choice, and the TCP usually resolves after several months (the maternal ab has to be catabolized by the infant’s system before they can fully recover)
  228. 228. Recognize that multiple sibs with neonatal TCP suggests isoimmune TCP
  229. 229. Know that TCP due to maternal ITP or isoimmune TCP usually resolves within 6-12 weeks
  230. 230. Plan the appropriate management of a pt with ITP
  231. 231. Do not give platelets as a standard, first line tx!!!
  232. 232. See above for other txes
  233. 233. Pancytopenia
  234. 234. Decreased Production
  235. 235. Understand that aplastic anemia and childhood leukemia may both present with purpura, pallor, and fever
  236. 236. Know that the absence of blasts in the peripheral blood of a patient with pancytopenia does not rule out the dx of leukemia
  237. 237. Coagulation Disorders
  238. 238. Congenital bleeding and thrombotic disorders
  239. 239. Know that excessive bleeding after circumcision may be the first sign of a congenital coagulation factor deficiency
  240. 240. Indentify prothrombin time and partial thromboplastin time as important parts of the evaluation of a pt with increased bruising
  241. 241. Prolonged PT = abnormal Factor VII
  242. 242. Prolonged PTT = abnormal Factor VIII, IX, XI
  243. 243. Both prolonged = abnormal Factor V, Factor X, prothrombin, fibrinogen
  244. 244. Know that a child born to the daughter of a person with hemophilia should be tested for that particular bleeding disorder
  245. 245. A child whose maternal grandparent has hemophilia needs to be checked
  246. 246. Carrier females have a 50% chance of passing the affected chromosome to male offspring
  247. 247. Know that HA is an important sx of intracranial bleeding and requires early assessment and tx
  248. 248. Understand that serious head trauma in a person with hemophilia requires careful assessment and early replacement tx even in the absence of neurologic abnormalities
  249. 249. Recognize bleeding in the forearm of a person with hemophilia as an emergency because of the danger of nerve compression
  250. 250. Know that femoral or jugular venipunctures should be avoided in the person with hemophilia who has not received replacement tx
  251. 251. Know that some kids with hemophilia have a negative family hx for bleeding disorders
  252. 252. Recognize that partial thromboplastin time is often normal in patients with vWF disease, but the bleeding time is commonly prolonged
  253. 253. Recognize that a manifestation of vWF in girls may be heavy menstrual bleeding
  254. 254. Recognize that a strong fam hx of PE or DVT may suggest a congenital hypercoagulable d/o
  255. 255. Understand the usefulness of DDAVP in the tx of a pt with hemophilia or vWF
  256. 256. DDAVP increases levels of vWF in the plasma and also causes a 2-4fold increase in factor VIII activity
  257. 257. Peak hours can be reached within one hour of treatment, making it useful for surgical prophylaxis
  258. 258. Acquired bleeding and thrombotic disorders
  259. 259. Identify the need for measuring PT, PTT and plt count as part of the eval for DIC in a child with sepsis and purpura
  260. 260. Prolonged PT/PTT
  261. 261. Decreased platelets
  262. 262. Decreased fibrinogen; serial measurement of fibrinogen is a sensitive and specific test for DIC
  263. 263. Increased FDP/d-dimer
  264. 264. Peripheral smear will show evidence of intravascular hemolysis, like fragmented, burr, or helmet shaped cells
  265. 265. Tx of DIC = find and treat the sause of the shock; acidosis and hypoxemia worsen DIC; may need to give platelets, cryoprecipitate or FFP to stop hemorrhaging.
  266. 266. Recognize purpura as an indication of bacterial sepsis in a febrile child
  267. 267. Neoplastic disorders
  268. 268. Hematologic malignancies
  269. 269. Recognize bone pain as a symptom of leukemia
  270. 270. Presentation with fever and bone pain is most common in younger children
  271. 271. The bone pain is due to marrow expansion and occurs in about 1/3 of kids with ALL
  272. 272. When presents with bone pain, may have an insidious onset with initially nl blood counts
  273. 273. Failure of NSAIDs and refusal to walk necessitate consideration of leukemia, particularly if the use of corticosteroids is considered
  274. 274. Understand that most patients with ALL will be cured of their disease using current tx strategies
  275. 275. 75-50% of kids with ALL and nearly 50% of kids with AML are cured
  276. 276. Identify the CNS and testes as important sites of relapse of ALL
  277. 277. CNS: all regimens now have prophylactic tx of the CNS because systemic chemo may not cross the BBB
  278. 278. Testes: therapy in males in prolonged for 1 year, which helps negate the risk of testicular recurrence
  279. 279. Identify Down syndrome as a disease with an increased risk of leukemia
  280. 280. Recognize the need to evaluation of supraclavicular LN enlargement
  281. 281. Worrisome and should prompt a throough eval for a cause, with malignancy high on the ddx
  282. 282. Low threshold for excisional bx
  283. 283. ID the CXR as an important part of the initial eval of the pt with unexplained LAD
  284. 284. Know that overwhelming sepsis is a serious complication in patients with Hodgkin dz who have undergone splenectomy, and know that such patients should be evaluated thoroughly if fever develops
  285. 285. Solid tumors
  286. 286. Understand that a neuroblastoma usually presents as a nontender abd mass
  287. 287. Solid, often fixed, painful or painless, irregularly shaped masses that cross the midline
  288. 288. Understand that urinary catecholamine excretion is increased in most patients with nboma and that tests for urinary VMA and HVA are appropriate screening tests for this kind of tumor
  289. 289. Know that Wilms tumor is associated with hemihypertrophy and/or aniridia
  290. 290. WAGR (Wilms, aniridia, GU abnormalities, Retardation)
  291. 291. Understand that Wilms usually presents as an abd mass and may cause htn and hematuria
  292. 292. Does NOT cross the midline (diff from nboma)
  293. 293. Htn in about 25% of cases, due to increase rennin activity
  294. 294. Understand the px of Wilms
  295. 295. Cure rate of about 85-90%
  296. 296. Recognize the si/sx of craniopharyngioma
  297. 297. Confined to sella tucica: usually clinical manifestations due to pituitary-hypothalamic involvement (short stature due to GH deficiency) and visual field defects due to pressure on optic chiasm,may also have other si of pituitary dysfxn like DI, hypothyroid, precocious puberty
  298. 298. If extands through the diaphragm sella, it may compress the third ventricle and cause sx of increased ICP like HA, vomiting and papilledma
  299. 299. Other sx can include alterations in personality, lethargy, hyperactivity, irritability, forgetfulness
  300. 300. Recognize the presenting si of brain tumor (eg, headache, deteriorating school performance, ataxia, emesis)
  301. 301. School age children: declining academic performance, fatigue, behavioral changes and vague HA, especially in the occipital or frontal region; over time the HA may be worse in the AM and lethargy and vomiting may develop; also horizontal diplopia and papilledema
  302. 302. Infants: irritability, anorexia, FTT, developmental regression can be signs. Macrocephaly, splitting of cranial sutures, bulging anterior fontanelle
  303. 303. “Setting sun” sign can be seen with attendant hydrocephalus (downward deviation of eyes)
  304. 304. Supratentotial lesions: hemiparesis, hemisensory loss, hyperreflexia, sz, visual complaints
  305. 305. Infratentorial: ataxia, hemiparesis, hyperreflexia, cranial neuropathies, but NOT seizures
  306. 306. Know that the presenting sx of osteosarcoma is usually bone pain or swelling
  307. 307. Systemic sx are rare
  308. 308. Occurs in the long bones near the growth plates
  309. 309. Identify the clinical and laboratory features of osteoid osteoma
  310. 310. Common benign bone tumor, occurs in those age 5-20 yrs (usually)
  311. 311. Usually present with gradually increasing sharp focal pain that is worse at the end of day or at night.
  312. 312. Pain usually no related to activity, it responds dramatically to NSAIDs
  313. 313. Normal lab findings
  314. 314. Plain films reveal a small spherical or round lytic lesion surrounded by soft tissue edema and reactive bone formation
  315. 315. Recognize the clinical manifestations of histiocytosis syndromes of childhood
  316. 316. Oncologic emergencies
  317. 317. Differentiate the clinical manifestations of spinal cord compression (eg, from a tumor) from those of other myelopathies and eval appropriately
  318. 318. Identify varicella as a life threatening illness in a pt receiving hemotx, and know that varicella Ig should be given immediately after exposure to varicella
  319. 319. Recognize the need for immediate eval of a febrile child who is neutropenic as a result of chemotx
  320. 320. Therapeutic considerations
  321. 321. Understand the gonadal effects associated with the use of chemotx drugs
  322. 322. Understand the effects of cranial irradiation on growth hormone secretion
  323. 323. Can diminish GH secretion, and replacement tx may be indicated
  324. 324. Be familiar with the late sequelae of cancer and cancer tx
  325. 325. Good tables in article, will try and copy into this