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Pediatrics > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q

Anemia Definition

A

Reduction in RBC mass or blood Hb concentration that is 2 standard deviations below the expected normal. for 6 mo -5 yo Hb less than 11.

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2
Q

Anemia Presentation

A

Acute: lethargy, tachycardia, pallor, irritability, poor oral intake. Jaundice (hemolytic): gallstones, petechia, purpura, ecchymosis, bleeding.

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3
Q

Fanconi Anemia Definition

A

Inherited bone marrow failure (aplastic anemia). Autosomal recessive. Bone marrow failure usually occurs less than 10 yo and effects all cell lines. Increased risk of malignancies.

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4
Q

Fanconi Anemia Presentation

A

Progressive pancytopenia. Congenital malformations: abnormal skin pigementation, short stature, absent or hypoplasia of the thumb/radius.

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5
Q

Fanconi Anemia Lab Findings

A

Present early with thrombocytopenia or leukopenia with is followed by anemia. Often misdiagnosed at ITP. BMB will show hypoplasia or aplasia.

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6
Q

Fanconi Anemia Treatment

A

Refer to hematology. Transfusions, infection prevention. HSCT is the definitive treatment.

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7
Q

Acquired Aplastic Anemia Definition

A

Peripheral pancytopenia with hypocellular bone marrow. Idiopathic or due to a trigger such as medications, toxins or viruses. Can lead to overwhelmng infection or severe hemorrhage.

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8
Q

Acquired Aplastic Anemia Lab Findings

A

Anemia, low WBC, thrombocytopenis, low reticulocytes.

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9
Q

Acquired Aplastic Anemia Treatment

A

Refer to hematology. Supportive therapy. HSCT.

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10
Q

Iron Deficiency Anemia Definition

A

Most common nutritional deficiency in kids especially from 6-24 mo. Most common in lower SES.

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11
Q

Iron Deficiency Anemia Presentation

A

Pallor, fatigue, irritability, pica, delayed motor development. May be asymptomatic.

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12
Q

Iron Deficiency Anemia Risks

A

Low SES, prematurity, lead exposure, exclusive breast feeders, feeding problems.

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13
Q

Iron Deficiency Anemia Screening

A

At 12 month visit. Also assess risk factors.

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14
Q

Iron Deficiency Anemia Lab Findings

A

Microcytic, hypochromic anemia. Ferritin less than 12.

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15
Q

Iron Deficiency Anemia Treatment

A

From Hb 10-11 monitor closely. Less than 10 treat with Iron supplementation of 6mg/kg/day divided into 3 doses.

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16
Q

Megaloblastic Anemias

A

Vitamin B12 deficiency and Folic acid deficiency

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17
Q

Vitamin B12 Deficiency Causes

A

Intestinal malabsorption or dietary insufficiency.

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18
Q

Folic Acid Deficiency Causes

A

Increase folate requirements during growth spurts or due to hemolytic anemia. Malabsorption, medications (methotrexate) or inadequate diet.

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19
Q

Megaloblastic Anemia Presentation

A

Pallor, glossitis. With Vitamin B 12 deficiency ther can be parasthesia, weakness, unsteady gate, decreased vibratory sensation and proprioception.

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20
Q

Megaloblastic Anemia Lab findings

A

Elevated MCV and MCH. Large neurtophils with hypersegmented nuclei. Macro-ovalocytes. Low levels of folic acid and/or B 12 (low metholonic acid).

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21
Q

Megaloblastic Anemia Treatmetn

A

Supplementation. Be sure to differentiate between folic acid and vitamin B 12 deficiency to avoid neuro deficits.

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22
Q

Hereditary Spherocytosis Definition

A

RBC membrane defect that causes them to be trapped in the spleen and destroyed.

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23
Q

Hereditary Spherocytosis Presentation

A

Hemolytic anemia, jaundice, gallstones, splenomegaly.

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24
Q

Hereditary Spherocytosis Lab Findings

A

Sphrerocytes with increased osmotic fragility.

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25
Q

Hereditary Spherocytosis Treatment

A

Splenectomy, suppotive care (+/- transfusion).

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26
Q

Thalassemia Lab Findings

A

Microcytic, hypochromic anemia. Hb electrophoresis is diagnositc.

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27
Q

Thalassemia Treatment

A

Supportive care, +/- transfusion, Iron monitorying, splenectomy, HSCT for severe beta-thalassemia.

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28
Q

Splenectomy

A

Try to wait until after 6 yo. Give H. Influenza, pneumococcal and meningococcal vaccines.

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29
Q

Sickle Cell Anemia Definition

A

Homozygous for Hb ss. RBC become sickle shaped with deoxygenated leading to vaso occlusion that causes severe pain and chronic hemolysis.

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30
Q

Sickle Cell Anemia Presentation

A

Anemia, splenomegaly (splenic infarcts can lead to asplenia)

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31
Q

Sickle Cell Anemia Lab Findings

A

Howel jolly bodies, target cells. Hb electrophoresis is diagnostic.

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32
Q

Sickle Cell Anemia Treatment

A

Supportive. Trigger avoidance. Pain management. Maintain hydration and proper oxygenation. Hydroxyurea. HSCT.

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33
Q

G6PD Definition

A

Genetic RBC enzyme defect that causes hemolytic anemia. The RBC can’t repair damage caused by oxidative stress. X-linked recessive. Most common in Africans, Mediterraneans and Asians.

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34
Q

G6PD Presentation

A

Episodic hemolysis when exposed to oxidative stress. Neonatal jaundice. Pallor, jaundice, hemoglobinuria.

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35
Q

G6PD Lab Findings

A

Heinz bodys, Bite-like deformity.

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36
Q

G6PD Treatment

A

Trigger avoidance and supportive care.

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37
Q

Lead Poisoning Lab Findings

A

Mild, hemolytic, normocytic anemia. Basophilic stippling.

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38
Q

Lead Poisoning Treatment

A

Chelation

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39
Q

Congenital Erythocytosis Definition

A

Increased RBC. Hv as high as 27.

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40
Q

Congenital Erythocytosis Presentation

A

Plethora, splenomegaly, HA and lethargy.

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41
Q

Congenital Erythocytosis Treatment

A

Phlebotomy

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42
Q

Secondary Polycythemia Definition

A

Polycythemia in response to hypoxemia from a cyanotic congenital heart diease or chronic pulmonary disease.

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43
Q

Secondary Polycythemia Treatment

A

Treat the underlying condition. Phlebotomy if indicated

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44
Q

Normal Platelet count

A

150,000-400,000 mm3. Spontaneous bleeding will occur at less than 20,000.

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45
Q

Prothrombin Time (PT)

A

Tests extrinisic and common pathways (I, II, V, VII, X, and tissue factor)

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46
Q

Activated Partial Thromboplastin Time (aPTT)

A

Tests the intrinsic and common pathways (I, II, V, VIII, IX, X, XI, XII)

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47
Q

INR (international normalized ratio)

A

A more accurate reflection of prothrombin time (PT).

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48
Q

Bleeding Time

A

Screens for platelet dysfunction and severe thrombocytopenia.

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49
Q

Idiopathic Thrombocytopenic Purpura (ITP) Definition

A

Immune mediated. Often follows a viral infection. Most common pediatric bleeding disorder in children 2-5 yo.

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50
Q

Idiopathic Thrombocytopenic Purpura (ITP) Presentation

A

Petechiae, ecchymosis, epistaxis

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51
Q

Idiopathic Thrombocytopenic Purpura (ITP) Lab Findings

A

Thrombocytopenia. Normal WBC, Hb, PT/aPTT. Is often a diagnosis of exclusion.

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52
Q

Idiopathic Thrombocytopenic Purpura (ITP) Treatment

A

90% resolve spontaneously. Avoid medications that compromise platelet function (NSAIDS, aspirin), bleeding percautions, prednisone. If sever then IVIG or splenectomy.

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53
Q

Von Willebrand Disease Definition

A

Most common inherited bleeding disorder. Autosomal dominant (m=f). Decreased level or impaired function of vWF so there is no platelet plug formation.

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54
Q

Von Willebrand Factor

A

Protein that binds factor VIII and is a cofactor for platelet adhesion to the endothelium.

55
Q

Von Willebrand Disease Presentation

A

Prolonged bleeding from the mucosa: epistaxis, menorrhagia, GI bleeding. Ecchymosis.

56
Q

Von Willebrand Disease Lab Findings

A

Prolonged bleeding time. Normal/Decreased factor VIII and vWF. Normal PT. Normal/Prolonged aPTT.

57
Q

Von Willebrand Disease Treatment

A

Desmopressin: releases vWF and factor VIII from endothelial stores. vWF replacement therapy.

58
Q

Hemophilia Definition

A

X-linked so more common in males. Hemophilia A is a deficiency in factor VIII and Hemophilia B is a deficiency in factor IX.

59
Q

Hemophilia Presentation

A

Bleeding due to impaired hemostasis not the platelet plug. Mild: bleed due to injury/surgery may not be apparent until later in life. Severe: Severe and spontaneous bleeding with an early age of onset. Bleeding can be delayed. Commonly bleed into joints and muscles (spontaneous hemarthrosis).

60
Q

Hemophilia Lab Findings

A

Normal platelet count, vWF, PT and bleeding time. Prolonged aPTT.

61
Q

Hemophilia Treatment

A

Desmopressin for Hemophilia A. Factor replacement until effective hemostasis is achieved.

62
Q

Disseminated Intravascular Coagulation (DIC) Definition

A

Hemorrhage and microvascular thrombosis usually triggered by an event such as sepsis, trauma or malignancy. The trigger activates the coagulation cascade which forms microthrombi and there is massive consumption of platelets, coag factors and fibrin which leads to severe bleeding.

63
Q

Disseminated Intravascular Coagulation (DIC) Presentation

A

Shock (end organ dysfunction). Diffuse bleeding: Hematuria, melena, purpura, petechiae, ooizng from needle punctures. Thrombotic lesions: purpura fulminans.

64
Q

Disseminated Intravascular Coagulation (DIC) Lab Findings

A

Decreased platelets. Prolonged aPTT/PT. Decreased fibrinogen. Elevated D-dimer and fibrin degradation products (FDP).

65
Q

Disseminated Intravascular Coagulation (DIC) Treatment

A

Treat underlying cause, replace coag factors, anticoagulation if indicated.

66
Q

Liver Disease

A

Causes bleeding disorder because it is the source of prothrombin, fibrinogen, and coag factors.

67
Q

Liver Disease Lab Findings

A

Normal/Low platelets. Prolonged PT/aPTT.

68
Q

Vitamin K Dependent factors

A

II, VII, IX and X

69
Q

Vitamin K Deficiency Lab Findings

A

Normal platelets with prolonged PT/aPTT.

70
Q

Protein C deficiency

A

Inherited Thrombotic Disorder. Homo/Heterozygous forms. Can develop warfarin induced skin necrosis.

71
Q

Protein C

A

Activated protein C inactivates factors V and VIII

72
Q

Protein S

A

Co factor for protein C

73
Q

Protein S deficiency

A

Inherited Thrombotic Disorder. Homo/Heterozygous forms.

74
Q

Factor V Leiden Mutation Definition

A

Inherited Thrombotic Disorder. Homo/Heterozygous forms. Factor V polymorphism (common point mutation) that makes it resistant to inactivation by protein C.

75
Q

Factor V Leiden Mutation Risks

A

High risk (2-7 fould increases) of VTE. Especially in homozygous form and with oral contraceptive use.

76
Q

Inherited Thrombotic Disorders Treatment

A

Anticoagulant prophylaxis (may be long term): UFH, LMWH (lovenox), warfarin. VTE episode is treated with anticoagulation for at least 3 mo.

77
Q

Henoch-Schonlein Purpura (HSP)

A

Small vessel vasculitis due to the deposition of IgA complexes. More common in males and 2-7 yo. Commonly preceded by a viral URI.

78
Q

Henoch-Schonlein Purpura (HSP) Presentation

A

Palpable purpura in lower extremitiy, arthritis/arthralgias, abdominal pain, hematuria.

79
Q

Henoch-Schonlein Purpura (HSP) Lab Findings

A

Normal/Elevated platelets. ASO titer can be elevated (strep A infection). Elevated IgA. occult blood. UA: hematuria and/or proteinuria.

80
Q

Henoch-Schonlein Purpura (HSP) Treatment

A

Supportive usually resolves spontaneously.

81
Q

Acute Lymphoblastic Leukemia (ALL) Definition

A

Uncontrolled proliferation of malignant lymphoid precursors. Most common pediatric cancer. Median age is 3-7 yo. Stains with TdT.

82
Q

Acute Myeloid Leukemia (AML) Definition

A

Uncontrolled proliferation of malignant myeloid precursors. More common in adults. Median age is 65 yo. AUER-RODS.

83
Q

Auer-rods

A

AML

84
Q

Cause of Leukemia

A

Radiation, chemo, tobacco. Myelodysplastic syndrome, paroxysmal nocturnal hematuria, myeloma. Down’s syndrome, neurofibromatosis, klinefelter syndrome. Familial. Human T-Cell leukemia virus.

85
Q

Leukemia Presentation

A

Pancytopenia with circulating blasts. Fatigue, ill appearing, bone pain. Rapid onset. Pallor, tachycardia, SOB. Ecchymosis, epistaxis. Fever, recurrent infections. Leukemia cutis. Inflitration of liver, spleen and lymph nodes. Leukostasis.

86
Q

Leukostasis

A

WBC count so high (immature cells) that is causes hyperviscosity. Dyspnea, HA, confusion. Treated with aggressive hydration and leukopharesis.

87
Q

ALL Specific Presentations

A

Gum hypertrophy, thymic mass, testicular infiltration, CNS involvement (HA and palsies).

88
Q

AML Specific Presentations

A

DIC.

89
Q

Leukemia Diagnostics

A

Bone marrow biopsy with greater than 20% blasts is diagnostic. With AML signs of translocation regardless of blast percentage.

90
Q

Leukemia Treatment

A

Aggressive hydration, blood transfusions PRN, Decrease uric acid levels (Allopurinol or rasburicase), +/- HSCT. AML=anthracycline and cytarabine). ALL=chemo.

91
Q

Tumor lysis syndrome

A

High potassium, uric acid and phosphate with decreased calcium and renal failure.

92
Q

Chronic Myeloid Leukemia (CML) Definition

A

Myeloproliferative neoplasm caused by dysregualtion and unregulated proliferation of mature granulocytes. Median age is 45-55. Due to translocation of 9 (ABL) and 22 (BCR) to form an oncogene that causes proliferation. Phases: chronic, accelerated, blast. Can lead to ALL or AML.

93
Q

CML Presentation

A

Fever, anorexia, early satiety, splenomegaly, weight loss, night sweats, dyspnea. Can be asymptomatic.

94
Q

CML Lab Findings

A

Elevated WBC +/- anemia. BCR/ABL gene on RT PCR.

95
Q

CML Treatment

A

Hydrea to reduce WBC count, tyrosine kinase inhibitors (gleevac, tasigna, bosulif), continued monitoring.

96
Q

Non-Hodgkins Lymphoma (NHL) Definition

A

Lymphoproliferative neoplasm from B-cell progenitors. 5th most common pediatric cancer. Aggressive in children. NO reed sternburg cells.

97
Q

Reed Sternburg Cells

A

Hodgkin’s Lymphoma

98
Q

Hodgkin’s Lymphoma (HL) Definition

A

Lymphoproliferative disorder with REED STERNBURG cells. Effects kids 15-19 yo. Associated with EBV exposure (cluster outbreaks).

99
Q

Lymphoma Presentation

A

Painless and persistent LAD. Hepatosplenomegaly. Night sweats, early satiety, pruritis. SVC syndrome, spinal cord compression, pericardial tamponade.

100
Q

NHL Specific Presentation

A

Primary mediastinal mass

101
Q

HL Specific Presentation

A

Alcohol induced painful LAD. Swollen supraclavicular nodes, mediastinal bulky mass.

102
Q

Lymphoma Staging

A

I: one area of nodes
II: two areas of nodes on the same side of the diaphragm
III: nodes on both sides of the diaphragm.
IV :involvement of extranodal sites (bone)
A: No B symptoms
B: Night sweats, weight loss, LAD
E: extranodal site contiguous with node

103
Q

Lymphoma diagnosis

A

Excision node biopsy is diagnostic. Elevated LDH/Uric acid. Beta 2 microglobulin. BMB for staging. 2D echo if using adriamycin. PFT is using bleomycin. Relapse=Rebiopsy.

104
Q

NHL Treatment

A

Chemo, allopurinol and supportive care.

105
Q

HL Treatment

A

Chemo +/- radiation. Relapse=HSCT. Higher risk of leukemia, other lymphomas and malignancies.

106
Q

Osteosarcoma Definition

A

Primary malignant tumor characterized by production of osteoid or immature bone by malignant cells. Children 15-19 yo due to growth spurt.

107
Q

Osteosarcoma Presentation

A

Usually in long bones (femur and tibia). Pain, swelling, fever, weight loss, malaise.

108
Q

Osteosarcoma Lab findings

A

Elevated alk phos and LDH. Biopsy is diagnostic.

109
Q

Osteosarcoma Treatment

A

Surgery, chemo +/- radiation.

110
Q

Ewing’s Sarcoma Definition

A

Tumor that develops in the bone (flat or long bones) or soft tissue. A spectrum of disorders that originate from common cell. Peak incidence is 10-15 yo. More common in males and is associated with urogenital abnormalities.

111
Q

Ewing’s Sarcoma Presentation

A

Pain, swelling +/- fever.

112
Q

Ewing’s Sarcoma diagnosis

A

Open biopsy by orthopedic oncologist.

113
Q

Ewing’s Sarcoma Treatmetn

A

Surgery, radiation, chemo (cytoxan + doxyrubicin +/- vincristine). Rate of relapse is high, suggests micrometastatic cells.

114
Q

Brain Tumor Presentation

A

Usually primary and glial in pediatrics. Increased ICP. AMS, seizures, syncope, papilledema, dysphagia, hemiparesis, palsies, nausea, vomiting, personality changes, FTT.

115
Q

Brain Tumor Diagnosis

A

MRI

116
Q

Brain Tumor Treatment

A

Surgery + chemo (temodar) +/- radiation. Relapse is common.

117
Q

Brain Tumor psuedoprogression

A

Follow up MRIs may have abnormalities due to damage from earlier treatment not actual progression.

118
Q

Neuroblastoma Definition

A

Large variation in presentation. Arise from primitive SNS ganglion. Usually occur on adrenal gland then abdomen then the thorax. Most common solid neoplasm in children. Often metastasize to the nodes, dura and orbits.

119
Q

Neuroblastoma Presentation

A

Abdominal mass, abdominal pain, proptosis, horner’s syndrome, fever, weight loss, bone pain, anemia, secretory diarrhea.

120
Q

Neuroblastoma Diagnosis

A

Biopsy

121
Q

Neuroblastoma Treatment

A

Low risk you can watch and wait. High risk needs surgery, chemo and radiation.

122
Q

Nephroblastoma Definition

A

4th most common peds tumor. Common in 15-19 yo. Bilateral presentation in younger children.

123
Q

Nephroblastoma Presentation

A

abdominal swelling, fever, hematuria, HTN.

124
Q

Nephroblastoma Diagnosis

A

CT or MRI

125
Q

Nephroblastoma Treatment

A

Resection

126
Q

Retinoblastoma Definition

A

Mutational inactivation of the tumor suppressor gene RB1. Genetically at risk for other malignancies.

127
Q

Retinoblastoma Presentation

A

Leukocoria

128
Q

Retinoblastoma Treatment

A

enucleation, external beam radiation and chemo. Often relapses within a year.

129
Q

Rhabdomyosarcoma Definition

A

Soft tissue sarcoma. More common in males. Arises from mesenchymal cells and can resemble fat, muscle or fibrous tissue. Younger kids get is in the head, neck and orbit. Older kids get it in the GU or extremities.

130
Q

Rhabdomyosarcoma Treatment

A

surgery, radiation, chemo

131
Q

Hepatic Tumors Definition

A

Can be primary tumors or mets from a nephroblastoma or Rhabdomyosarcoma. Usually found in kids less than 5 yo.

132
Q

Hepatic Tumor diagnosis

A

CT or MRI

133
Q

Hepatic Tumor Treatment

A

Resection

Pediatrics (8 decks)

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