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Peds Heme Flashcards

1
Q

Lymphoid malignancies

A

Lymphoma is the most common subtype of the hematologic malignancies and is heralded by LAD
3rd most common malignancy in childhood
Main categories: Non-Hodgkin
Hodgkin lymphoma

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2
Q

Epidemiology and risk factors for lymphoid malignancies

A

NHL occurs more in men, incidence increases with age
Hodgkin lymphoma has a bimodal age distribution (15-45 yrs and after age 55 yrs)
Mucosa-associated lymphoid tissue (MALT) lymphoma is caused by an underlying infection of H. pylori
Other infections: EBV, HIV, HTLV-1, Hep B, Hep C

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3
Q

Evaluation and dx of lymphoid malignancies- hx

A

Fever
Night Sweats
Weight loss
B sx

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4
Q

Evaluation and dx of lymphoid malignancies- PE

A

Number of sites
Size
Consistency
Persistent or enlarging LAD

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5
Q

Evaluation and dx of lymphoid malignancies- labs

A 
CXR
CBC with differential
ESR
CMP
Bx
-Fine needle aspiration: no LAD architecture
-Core bx/excisional bx: LN architecture
Biopsy specimen
Secondary testing for initial staging
Total body PET/CT scan and iliac crest bone marrow bx to complete staging
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6
Q

Biopsy specimen for lymphoid malignancies

A

Histopathologic- test you must begin with if you suspect lymphoma
Cytogenic
Fluorescence in situ hybridization (FISH)
Immunophenotypic analysis
Gene expression profiling

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7
Q

Classification, staging, and prognosis of malignant lymphoma

A

NHL has >20 subtypes
-Cell surface antigen expression
-B cell (85%), T cell (13%), or NK (2%) immunophenotype
Standard uptake value
-Indicator of glucose uptake and metabolism
3 prognostic groups
-Indolent, aggressive, and highly agressive

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8
Q

Follicular lymphoma

A 
20% of all NHL cases (70% of indolent)
Small cells (CD10, 19, 20, 22)
Translocation t(14:18) that causes an overexpression of the BCL2 oncogene
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9
Q

Tx of follicular lymphoma

A

Local-Rituximab and involved-field radiation therapy
Systemic- riuximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone
Hateopoietic stem cell transplant- younger pts

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10
Q

MALT

A

Originates in B cells in GI tissue
CD 20 surface antigen
Usually caused by chronic inflammation of an ulcer bed from H. pylori
-Complete remissions achieved in 70% of pts with antimicrobial therapy and concomitant PPI tx
Localized therapy or chemo

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11
Q

Diffuse Large B-Cell Lymphoma (DLBCL)

A 
MC form of lymphoma (30%)
Usually presents with advanced dz
Most aggressive forms
-Burkitt lymphoma
-Lymphoblastic lymphoma
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12
Q

Tx for diffuse large b-cell lymphoma (DLBCL)

A

Standard therapy: R-CHOP
High response rates (80%)
Curative in nearly 50% of pts

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13
Q

What are the types of indolent lymphomas?

A

Follicular lymphoma

MALT

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14
Q

Types of aggressive lymphomas

A

Diffuse large b-cell lymphoma (DLBCL)
Mantle cell lymphoma
Cutaneous t-cell NHL
Hodgkin lymphoma

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15
Q

Mantle cell lymphoma

A

Rare form of NHL

Most present with advanced dz

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16
Q

Cutaneous T-cell NHL

A

CD4 cells infiltrate skin and cause rash
-Raised plaques, diffuse skin erythema, skin ulcers
Organ infiltration, immunodeficiency, recurrent bacterial infections
Looks like dermatitis if you don’t know better

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17
Q

Tx of cutaneous T-cell NHL

A

Stage I and II (skin only)- median survival >20 yrs
Topical glucocorticoids
+/- retinoids/PUVA/interferon alfa

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18
Q

What are the four classic histologic subtypes?

A

Nodular sclerosing
Mixed cellularity
Lymphocyte predominant
Lymphocyte depleted

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19
Q

What is seen under the microscope in Hodgkin lymphoma?

A

Reed-Sternberg cells that have an owl-eyed appearance

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20
Q

What can cause a mediastinal mass in the anterior mediastinum?

A

Thymoma
Teratoma
Thyroid cancer
Parathyroid cancer

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21
Q

Presentation of Hodgkin lymphoma

A

Palpable, firm lymph nodes, +/- B sx
Splenomegaly- 30%
Hepatomegaly- 5%

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22
Q

Tx of Hodgkin lymphoma

A

Stage I and II dz- radiation +/- chemo (short course)
Stage III and IV dz- full chemo
-Doxyrubicin, bleomycin, vinblastine, decarbazine
-Rituximab is added in CD20-pos dz

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23
Q

Chemo toxicities in Hodgkin lymphoma

A

Bleomycin-induced pneumonitis
Doxorubicin-induced cardiac dysfunction
Vincristine-induced neuropathy

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24
Q

Prognosis of NHL

A

Related to stage

3-yr survival rates 70-90%

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25
Prognosis of Hodkin lymphom
90% 5-yr survival rate
26
Genetic factors of leukemia
+/- chromosomal translocations | Reports of familial leukemia
27
Predisposing constitutional genotypes to leukemia
Down syndrome Fanconi anemia Neurofibromatosis 1
28
Environmental factors to leukemia
Ionizing radiation | Certain chemo agents
29
S/sx of leukemia
``` Bone marrow failure Specific tissue infiltration -LN, liver, spleen, brain, bone, skin, gingiva, testes Fever Pallor Petechiae Ecchymoses Lethargy Malaise Anorexia Bone or joint pain ```
30
Chronic myelogenous leukemia
Myeloproliferative disorder Caused by the Philadelphia chromosome -Translocation of chromosomes 9 and 22 -Fusion gene called BCR-ABL
31
S/sx of chronic myelogenous leukemia
Constiutional sx | Splenomegaly
32
Lab findings of chronic myelogenous leukemia
Granulocytic leukocytosis usually >50K Peripheral blood -Reverse transcriptase polymerase chain reaction -Fluorescence in situ hybridization (FISH)
33
What chronic myelogenous leukemia looks like under the microscope
Lots of different types- sign of rushed proliferation
34
Tx of chronic myelogenous leukemia
``` Tyrosine-kinase inhibitors (TKI) -Imatinib (1st gen) -Improved 8-yr survival from 50% to 87% -QT prolongation Allogenic hematopoietic stem cell transplantation (rare) ```
35
Chronic lymphocytic leukemia
``` B-cell CLL is MC form of adult leukemia Median age of 70 yrs Often asymptomatic at presentation More aggressive in younger pts No definitive causative factors Dx by flow cytometry (CD5 and CD23) Prognosis determined by gene mutation status -Worse if chromosome 17p deletion found ```
36
What can be seen under the microscope in CLL?
Some nl mixed with the leukocytes SMUDGE cells They are pathognomonic for CLL
37
Components of concomitant CLL
Autoimmune dz Recurrent infections Secondary malignancies
38
Autoimmune dz in concomitant CLL
ITP | Hemolytic anemia
39
Recurrent infections in CLL
Sinus Pulmonary +/- low serum IgG levels requiring replacement therapy
40
Tx of CLL
Goal is usually palliation Combo therapy -Rituximab and multiagent chemo --Usually with Fludarabine and cyclosphamide Allogenic HSCT is reserved for younger pts with aggressive symptomatic dz
41
Acute Myelogenous Leukemia
An acquired, malignant HSC disorder that may manifest de novo from preceding MDS (myelodysplastic syndromes) or MPN (myeloproliferative neoplasms) - >20% myeloblasts in peripheral blood or bone marrow - RAPID blast division- STAT consult
42
Findings of acute myelogenous leukemia
Anemia Neutropenia Thrombocytopenia Leukapharesis is a temporizing measure
43
What is the mean age at presentation of acute myelogenous leukemia?
67, but can occur in kids
44
What can be seen in microscopy in acute myelogenous leukemia
Auer rod
45
Lab findings of acute myelogenous leukemia
Pancytopenia or leukocytosis with circulating blasts Auer rods DIC if promyelocytic leukemia
46
Tx of acute myelogenous leukemia
Induction chemo
47
Acute lymphoblastic leukemia
Seen more commonly in childhood -75% of all childhood leukemias -Incidence peaks at 2-5 yrs (boys>girls) >25% lymphoblasts in blood or bone marrow Frequently involves the CNS requiring staging by CSF and prophylaxis with intrathecal chemo
48
Predictors of poor outcome for acute lymphoblastic leukemia
``` Advanced age B-cell vs T-cell dz More than 30K blasts at dx Poor-risk cytogenetics Philidelphia chromosome also portends poor prognosis but now offers an attractive target to the TKI dasatinib (2nd gen) ```
49
Tx of acute lymphoblastic leukemia
Chemo with L-asparaginase -Induction, Intensification, 2 yrs of maintenance therapy -Prognosis is better in children than adults --80% cure rate for childhood ALL --30-40% of adult ALL cured by chemo Hematopoietic stem cell transplantation
50
Primary hemostasis
Injured vessel wall Von Willebrand factor Platelets
51
Hx with bleeding disorders
FHx of bleeding | Meds-NSAIDs
52
PE of bleeding disorders
Petechiae, ecchymoses, telangiectasias Splenomegaly Liver dz- jaundice, spider angioma
53
Mechanism tested with PT
Extrinsic and common pathway
54
Nl values of PT
<12 sec beyond neonate | 12-18 sec term neonate
55
Disorder of PT
``` Defect in vit K-dependent factors Hemorrhagic dz of newborn Malabsorption Liver dz DIC Oral anticoags Ingestion of rat poison ```
56
aPTT mechanism tested
Intrinsic and common pathway
57
aPTT nl values
25-40 sec beyond neonate | 70 sec in term neonate
58
Disorders of aPTT
``` Hemophilia vWB diz Heparin DEC Deficient factors 12 and 11 Lupus anticoagulant ```
59
Thrombin time- mechanism tested
Fibrinogen to fibrin conversion
60
Nl thrombin time values
10-15 sec beyond neonate | 12-17 sec in term neonate
61
Thrombin time disorders
``` Fibrin split products DIC Hypofibrinogemia Heparin Uremia ```
62
Bleeding time mechanism tested
Hemostasis, capillary and platelet function
63
Nl values of bleeding time
3-7 min beyond neonate
64
Disorders of bleeding time
Platelet dysfunction Thrombocytopenia vWB dz Aspirin
65
Platelet count mechanism tested
Platelet number
66
Nl platelet values
150K-450K/mL
67
Platelet disorders
Thombocytopenia differential dx
68
PFA mechanism tested
Closure time
69
Blood smear mechanism tested
Platelet number and size | RBC morphology
70
Blood smear disorders detected
Large platelets suggest peripheral destruction | Fragmented, bizarre RBC morphology suggests microangiopathic process (e.g, hemolytic uremic syndrome, hemangioma, DIC)
71
X-linked recessive bleeding disorders
Hemophilia A- factor VIII deficiency (1 in 5000) | Hemophilia B- factor IX deficiency (1 in 25K)
72
Clinical manifestations of X-linked recessive bleeding disorders
Spontaneous bleeding episodes Hemarthrosis -Ankle, knee, elbow
73
Lab findings of hemophilia A and B
Prolongation of aPTT | Nl PT
74
Tx of hemophilia A and B
Desmopressin (DDAVP)- Hemophilia A (mild) | Factor concentrates- Hemophilia A or B
75
Von Willebrand dz
``` The MC inherited bleeding disorder (autosomal dominant) Type I (80%)- quantitative ```
76
Clinical manifestations of von Willebrand dz
Mucocutaneous bleeding | Nosebleeds, easy bruising, bleeding gums
77
Labs for vWB disease
vWF antigen measured functionally in the ristocetin cofactor assay
78
Tx of vWB dz
Desmopressin vWF-containing concentrate Vaccine
79
DIC
Abnormal coagulation and fibrinolysis Usually associated with shock/sepsis Coagulation factors are consumed as are anticoagulant proteins
80
Lab findings of DIC
Decreased platelets and fibrinogen Prolonged PT, aPTT Elevated D-dimer
81
Tx of DIC
Treat the disorder including DIC first | Support the pt (hypoxia, acidosis, factors, platelets, etc)

Peds (11 decks)

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