Heme/Onc Flashcards
(133 cards)
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.
2
Q
Anemia Presentation
A
Acute: lethargy, tachycardia, pallor, irritability, poor oral intake. Jaundice (hemolytic): gallstones, petechia, purpura, ecchymosis, bleeding.
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.
4
Q
Fanconi Anemia Presentation
A
Progressive pancytopenia. Congenital malformations: abnormal skin pigementation, short stature, absent or hypoplasia of the thumb/radius.
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.
6
Q
Fanconi Anemia Treatment
A
Refer to hematology. Transfusions, infection prevention. HSCT is the definitive treatment.
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.
8
Q
Acquired Aplastic Anemia Lab Findings
A
Anemia, low WBC, thrombocytopenis, low reticulocytes.
9
Q
Acquired Aplastic Anemia Treatment
A
Refer to hematology. Supportive therapy. HSCT.
10
Q
Iron Deficiency Anemia Definition
A
Most common nutritional deficiency in kids especially from 6-24 mo. Most common in lower SES.
11
Q
Iron Deficiency Anemia Presentation
A
Pallor, fatigue, irritability, pica, delayed motor development. May be asymptomatic.
12
Q
Iron Deficiency Anemia Risks
A
Low SES, prematurity, lead exposure, exclusive breast feeders, feeding problems.
13
Q
Iron Deficiency Anemia Screening
A
At 12 month visit. Also assess risk factors.
14
Q
Iron Deficiency Anemia Lab Findings
A
Microcytic, hypochromic anemia. Ferritin less than 12.
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.
16
Q
Megaloblastic Anemias
A
Vitamin B12 deficiency and Folic acid deficiency
17
Q
Vitamin B12 Deficiency Causes
A
Intestinal malabsorption or dietary insufficiency.
18
Q
Folic Acid Deficiency Causes
A
Increase folate requirements during growth spurts or due to hemolytic anemia. Malabsorption, medications (methotrexate) or inadequate diet.
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.
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).
21
Q
Megaloblastic Anemia Treatmetn
A
Supplementation. Be sure to differentiate between folic acid and vitamin B 12 deficiency to avoid neuro deficits.
22
Q
Hereditary Spherocytosis Definition
A
RBC membrane defect that causes them to be trapped in the spleen and destroyed.
23
Q
Hereditary Spherocytosis Presentation
A
Hemolytic anemia, jaundice, gallstones, splenomegaly.
24
Q
Hereditary Spherocytosis Lab Findings
A
Sphrerocytes with increased osmotic fragility.
25
Hereditary Spherocytosis Treatment
Splenectomy, suppotive care (+/- transfusion).
26
Thalassemia Lab Findings
Microcytic, hypochromic anemia. Hb electrophoresis is diagnositc.
27
Thalassemia Treatment
Supportive care, +/- transfusion, Iron monitorying, splenectomy, HSCT for severe beta-thalassemia.
28
Splenectomy
Try to wait until after 6 yo. Give H. Influenza, pneumococcal and meningococcal vaccines.
29
Sickle Cell Anemia Definition
Homozygous for Hb ss. RBC become sickle shaped with deoxygenated leading to vaso occlusion that causes severe pain and chronic hemolysis.
30
Sickle Cell Anemia Presentation
Anemia, splenomegaly (splenic infarcts can lead to asplenia)
31
Sickle Cell Anemia Lab Findings
Howel jolly bodies, target cells. Hb electrophoresis is diagnostic.
32
Sickle Cell Anemia Treatment
Supportive. Trigger avoidance. Pain management. Maintain hydration and proper oxygenation. Hydroxyurea. HSCT.
33
G6PD Definition
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.
34
G6PD Presentation
Episodic hemolysis when exposed to oxidative stress. Neonatal jaundice. Pallor, jaundice, hemoglobinuria.
35
G6PD Lab Findings
Heinz bodys, Bite-like deformity.
36
G6PD Treatment
Trigger avoidance and supportive care.
37
Lead Poisoning Lab Findings
Mild, hemolytic, normocytic anemia. Basophilic stippling.
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Lead Poisoning Treatment
Chelation
39
Congenital Erythocytosis Definition
Increased RBC. Hv as high as 27.
40
Congenital Erythocytosis Presentation
Plethora, splenomegaly, HA and lethargy.
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Congenital Erythocytosis Treatment
Phlebotomy
42
Secondary Polycythemia Definition
Polycythemia in response to hypoxemia from a cyanotic congenital heart diease or chronic pulmonary disease.
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Secondary Polycythemia Treatment
Treat the underlying condition. Phlebotomy if indicated
44
Normal Platelet count
150,000-400,000 mm3. Spontaneous bleeding will occur at less than 20,000.
45
Prothrombin Time (PT)
Tests extrinisic and common pathways (I, II, V, VII, X, and tissue factor)
46
Activated Partial Thromboplastin Time (aPTT)
Tests the intrinsic and common pathways (I, II, V, VIII, IX, X, XI, XII)
47
INR (international normalized ratio)
A more accurate reflection of prothrombin time (PT).
48
Bleeding Time
Screens for platelet dysfunction and severe thrombocytopenia.
49
Idiopathic Thrombocytopenic Purpura (ITP) Definition
Immune mediated. Often follows a viral infection. Most common pediatric bleeding disorder in children 2-5 yo.
50
Idiopathic Thrombocytopenic Purpura (ITP) Presentation
Petechiae, ecchymosis, epistaxis
51
Idiopathic Thrombocytopenic Purpura (ITP) Lab Findings
Thrombocytopenia. Normal WBC, Hb, PT/aPTT. Is often a diagnosis of exclusion.
52
Idiopathic Thrombocytopenic Purpura (ITP) Treatment
90% resolve spontaneously. Avoid medications that compromise platelet function (NSAIDS, aspirin), bleeding percautions, prednisone. If sever then IVIG or splenectomy.
53
Von Willebrand Disease Definition
Most common inherited bleeding disorder. Autosomal dominant (m=f). Decreased level or impaired function of vWF so there is no platelet plug formation.
54
Von Willebrand Factor
Protein that binds factor VIII and is a cofactor for platelet adhesion to the endothelium.
55
Von Willebrand Disease Presentation
Prolonged bleeding from the mucosa: epistaxis, menorrhagia, GI bleeding. Ecchymosis.
56
Von Willebrand Disease Lab Findings
Prolonged bleeding time. Normal/Decreased factor VIII and vWF. Normal PT. Normal/Prolonged aPTT.
57
Von Willebrand Disease Treatment
Desmopressin: releases vWF and factor VIII from endothelial stores. vWF replacement therapy.
58
Hemophilia Definition
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
Hemophilia Presentation
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
Hemophilia Lab Findings
Normal platelet count, vWF, PT and bleeding time. Prolonged aPTT.
61
Hemophilia Treatment
Desmopressin for Hemophilia A. Factor replacement until effective hemostasis is achieved.
62
Disseminated Intravascular Coagulation (DIC) Definition
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
Disseminated Intravascular Coagulation (DIC) Presentation
Shock (end organ dysfunction). Diffuse bleeding: Hematuria, melena, purpura, petechiae, ooizng from needle punctures. Thrombotic lesions: purpura fulminans.
64
Disseminated Intravascular Coagulation (DIC) Lab Findings
Decreased platelets. Prolonged aPTT/PT. Decreased fibrinogen. Elevated D-dimer and fibrin degradation products (FDP).
65
Disseminated Intravascular Coagulation (DIC) Treatment
Treat underlying cause, replace coag factors, anticoagulation if indicated.
66
Liver Disease
Causes bleeding disorder because it is the source of prothrombin, fibrinogen, and coag factors.
67
Liver Disease Lab Findings
Normal/Low platelets. Prolonged PT/aPTT.
68
Vitamin K Dependent factors
II, VII, IX and X
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Vitamin K Deficiency Lab Findings
Normal platelets with prolonged PT/aPTT.
70
Protein C deficiency
Inherited Thrombotic Disorder. Homo/Heterozygous forms. Can develop warfarin induced skin necrosis.
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Protein C
Activated protein C inactivates factors V and VIII
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Protein S
Co factor for protein C
73
Protein S deficiency
Inherited Thrombotic Disorder. Homo/Heterozygous forms.
74
Factor V Leiden Mutation Definition
Inherited Thrombotic Disorder. Homo/Heterozygous forms. Factor V polymorphism (common point mutation) that makes it resistant to inactivation by protein C.
75
Factor V Leiden Mutation Risks
High risk (2-7 fould increases) of VTE. Especially in homozygous form and with oral contraceptive use.
76
Inherited Thrombotic Disorders Treatment
Anticoagulant prophylaxis (may be long term): UFH, LMWH (lovenox), warfarin. VTE episode is treated with anticoagulation for at least 3 mo.
77
Henoch-Schonlein Purpura (HSP)
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
Henoch-Schonlein Purpura (HSP) Presentation
Palpable purpura in lower extremitiy, arthritis/arthralgias, abdominal pain, hematuria.
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Henoch-Schonlein Purpura (HSP) Lab Findings
Normal/Elevated platelets. ASO titer can be elevated (strep A infection). Elevated IgA. occult blood. UA: hematuria and/or proteinuria.
80
Henoch-Schonlein Purpura (HSP) Treatment
Supportive usually resolves spontaneously.
81
Acute Lymphoblastic Leukemia (ALL) Definition
Uncontrolled proliferation of malignant lymphoid precursors. Most common pediatric cancer. Median age is 3-7 yo. Stains with TdT.
82
Acute Myeloid Leukemia (AML) Definition
Uncontrolled proliferation of malignant myeloid precursors. More common in adults. Median age is 65 yo. AUER-RODS.
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Auer-rods
AML
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Cause of Leukemia
Radiation, chemo, tobacco. Myelodysplastic syndrome, paroxysmal nocturnal hematuria, myeloma. Down's syndrome, neurofibromatosis, klinefelter syndrome. Familial. Human T-Cell leukemia virus.
85
Leukemia Presentation
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
Leukostasis
WBC count so high (immature cells) that is causes hyperviscosity. Dyspnea, HA, confusion. Treated with aggressive hydration and leukopharesis.
87
ALL Specific Presentations
Gum hypertrophy, thymic mass, testicular infiltration, CNS involvement (HA and palsies).
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AML Specific Presentations
DIC.
89
Leukemia Diagnostics
Bone marrow biopsy with greater than 20% blasts is diagnostic. With AML signs of translocation regardless of blast percentage.
90
Leukemia Treatment
Aggressive hydration, blood transfusions PRN, Decrease uric acid levels (Allopurinol or rasburicase), +/- HSCT. AML=anthracycline and cytarabine). ALL=chemo.
91
Tumor lysis syndrome
High potassium, uric acid and phosphate with decreased calcium and renal failure.
92
Chronic Myeloid Leukemia (CML) Definition
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
CML Presentation
Fever, anorexia, early satiety, splenomegaly, weight loss, night sweats, dyspnea. Can be asymptomatic.
94
CML Lab Findings
Elevated WBC +/- anemia. BCR/ABL gene on RT PCR.
95
CML Treatment
Hydrea to reduce WBC count, tyrosine kinase inhibitors (gleevac, tasigna, bosulif), continued monitoring.
96
Non-Hodgkins Lymphoma (NHL) Definition
Lymphoproliferative neoplasm from B-cell progenitors. 5th most common pediatric cancer. Aggressive in children. NO reed sternburg cells.
97
Reed Sternburg Cells
Hodgkin's Lymphoma
98
Hodgkin's Lymphoma (HL) Definition
Lymphoproliferative disorder with REED STERNBURG cells. Effects kids 15-19 yo. Associated with EBV exposure (cluster outbreaks).
99
Lymphoma Presentation
Painless and persistent LAD. Hepatosplenomegaly. Night sweats, early satiety, pruritis. SVC syndrome, spinal cord compression, pericardial tamponade.
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NHL Specific Presentation
Primary mediastinal mass
101
HL Specific Presentation
Alcohol induced painful LAD. Swollen supraclavicular nodes, mediastinal bulky mass.
102
Lymphoma Staging
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
Lymphoma diagnosis
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.
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NHL Treatment
Chemo, allopurinol and supportive care.
105
HL Treatment
Chemo +/- radiation. Relapse=HSCT. Higher risk of leukemia, other lymphomas and malignancies.
106
Osteosarcoma Definition
Primary malignant tumor characterized by production of osteoid or immature bone by malignant cells. Children 15-19 yo due to growth spurt.
107
Osteosarcoma Presentation
Usually in long bones (femur and tibia). Pain, swelling, fever, weight loss, malaise.
108
Osteosarcoma Lab findings
Elevated alk phos and LDH. Biopsy is diagnostic.
109
Osteosarcoma Treatment
Surgery, chemo +/- radiation.
110
Ewing's Sarcoma Definition
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
Ewing's Sarcoma Presentation
Pain, swelling +/- fever.
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Ewing's Sarcoma diagnosis
Open biopsy by orthopedic oncologist.
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Ewing's Sarcoma Treatmetn
Surgery, radiation, chemo (cytoxan + doxyrubicin +/- vincristine). Rate of relapse is high, suggests micrometastatic cells.
114
Brain Tumor Presentation
Usually primary and glial in pediatrics. Increased ICP. AMS, seizures, syncope, papilledema, dysphagia, hemiparesis, palsies, nausea, vomiting, personality changes, FTT.
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Brain Tumor Diagnosis
MRI
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Brain Tumor Treatment
Surgery + chemo (temodar) +/- radiation. Relapse is common.
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Brain Tumor psuedoprogression
Follow up MRIs may have abnormalities due to damage from earlier treatment not actual progression.
118
Neuroblastoma Definition
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
Neuroblastoma Presentation
Abdominal mass, abdominal pain, proptosis, horner's syndrome, fever, weight loss, bone pain, anemia, secretory diarrhea.
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Neuroblastoma Diagnosis
Biopsy
121
Neuroblastoma Treatment
Low risk you can watch and wait. High risk needs surgery, chemo and radiation.
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Nephroblastoma Definition
4th most common peds tumor. Common in 15-19 yo. Bilateral presentation in younger children.
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Nephroblastoma Presentation
abdominal swelling, fever, hematuria, HTN.
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Nephroblastoma Diagnosis
CT or MRI
125
Nephroblastoma Treatment
Resection
126
Retinoblastoma Definition
Mutational inactivation of the tumor suppressor gene RB1. Genetically at risk for other malignancies.
127
Retinoblastoma Presentation
Leukocoria
128
Retinoblastoma Treatment
enucleation, external beam radiation and chemo. Often relapses within a year.
129
Rhabdomyosarcoma Definition
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
Rhabdomyosarcoma Treatment
surgery, radiation, chemo
131
Hepatic Tumors Definition
Can be primary tumors or mets from a nephroblastoma or Rhabdomyosarcoma. Usually found in kids less than 5 yo.
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Hepatic Tumor diagnosis
CT or MRI
133
Hepatic Tumor Treatment
Resection
