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Peds > Peds Heme > Flashcards

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
Q

Prognosis of Hodkin lymphom

A

90% 5-yr survival rate

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26
Q

Genetic factors of leukemia

A

+/- chromosomal translocations

Reports of familial leukemia

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27
Q

Predisposing constitutional genotypes to leukemia

A

Down syndrome
Fanconi anemia
Neurofibromatosis 1

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28
Q

Environmental factors to leukemia

A

Ionizing radiation

Certain chemo agents

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29
Q

S/sx of leukemia

A 
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
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30
Q

Chronic myelogenous leukemia

A

Myeloproliferative disorder
Caused by the Philadelphia chromosome
-Translocation of chromosomes 9 and 22
-Fusion gene called BCR-ABL

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31
Q

S/sx of chronic myelogenous leukemia

A

Constiutional sx

Splenomegaly

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32
Q

Lab findings of chronic myelogenous leukemia

A

Granulocytic leukocytosis usually >50K
Peripheral blood
-Reverse transcriptase polymerase chain reaction
-Fluorescence in situ hybridization (FISH)

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33
Q

What chronic myelogenous leukemia looks like under the microscope

A

Lots of different types- sign of rushed proliferation

34
Q

Tx of chronic myelogenous leukemia

A 
Tyrosine-kinase inhibitors (TKI)
-Imatinib (1st gen)
-Improved 8-yr survival from 50% to 87%
-QT prolongation
Allogenic hematopoietic stem cell transplantation (rare)
35
Q

Chronic lymphocytic leukemia

A 
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
Q

What can be seen under the microscope in CLL?

A

Some nl mixed with the leukocytes
SMUDGE cells
They are pathognomonic for CLL

37
Q

Components of concomitant CLL

A

Autoimmune dz
Recurrent infections
Secondary malignancies

38
Q

Autoimmune dz in concomitant CLL

A

ITP

Hemolytic anemia

39
Q

Recurrent infections in CLL

A

Sinus
Pulmonary
+/- low serum IgG levels requiring replacement therapy

40
Q

Tx of CLL

A

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
Q

Acute Myelogenous Leukemia

A

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
Q

Findings of acute myelogenous leukemia

A

Anemia
Neutropenia
Thrombocytopenia
Leukapharesis is a temporizing measure

43
Q

What is the mean age at presentation of acute myelogenous leukemia?

A

67, but can occur in kids

44
Q

What can be seen in microscopy in acute myelogenous leukemia

A

Auer rod

45
Q

Lab findings of acute myelogenous leukemia

A

Pancytopenia or leukocytosis with circulating blasts
Auer rods
DIC if promyelocytic leukemia

46
Q

Tx of acute myelogenous leukemia

A

Induction chemo

47
Q

Acute lymphoblastic leukemia

A

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
Q

Predictors of poor outcome for acute lymphoblastic leukemia

A 
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
Q

Tx of acute lymphoblastic leukemia

A

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
Q

Primary hemostasis

A

Injured vessel wall
Von Willebrand factor
Platelets

51
Q

Hx with bleeding disorders

A

FHx of bleeding

Meds-NSAIDs

52
Q

PE of bleeding disorders

A

Petechiae, ecchymoses, telangiectasias
Splenomegaly
Liver dz- jaundice, spider angioma

53
Q

Mechanism tested with PT

A

Extrinsic and common pathway

54
Q

Nl values of PT

A

<12 sec beyond neonate

12-18 sec term neonate

55
Q

Disorder of PT

A 
Defect in vit K-dependent factors
Hemorrhagic dz of newborn
Malabsorption
Liver dz
DIC
Oral anticoags
Ingestion of rat poison
56
Q

aPTT mechanism tested

A

Intrinsic and common pathway

57
Q

aPTT nl values

A

25-40 sec beyond neonate

70 sec in term neonate

58
Q

Disorders of aPTT

A 
Hemophilia
vWB diz
Heparin
DEC
Deficient factors 12 and 11
Lupus anticoagulant
59
Q

Thrombin time- mechanism tested

A

Fibrinogen to fibrin conversion

60
Q

Nl thrombin time values

A

10-15 sec beyond neonate

12-17 sec in term neonate

61
Q

Thrombin time disorders

A 
Fibrin split products
DIC
Hypofibrinogemia
Heparin
Uremia
62
Q

Bleeding time mechanism tested

A

Hemostasis, capillary and platelet function

63
Q

Nl values of bleeding time

A

3-7 min beyond neonate

64
Q

Disorders of bleeding time

A

Platelet dysfunction
Thrombocytopenia
vWB dz
Aspirin

65
Q

Platelet count mechanism tested

A

Platelet number

66
Q

Nl platelet values

A

150K-450K/mL

67
Q

Platelet disorders

A

Thombocytopenia differential dx

68
Q

PFA mechanism tested

A

Closure time

69
Q

Blood smear mechanism tested

A

Platelet number and size

RBC morphology

70
Q

Blood smear disorders detected

A

Large platelets suggest peripheral destruction

Fragmented, bizarre RBC morphology suggests microangiopathic process (e.g, hemolytic uremic syndrome, hemangioma, DIC)

71
Q

X-linked recessive bleeding disorders

A

Hemophilia A- factor VIII deficiency (1 in 5000)

Hemophilia B- factor IX deficiency (1 in 25K)

72
Q

Clinical manifestations of X-linked recessive bleeding disorders

A

Spontaneous bleeding episodes
Hemarthrosis
-Ankle, knee, elbow

73
Q

Lab findings of hemophilia A and B

A

Prolongation of aPTT

Nl PT

74
Q

Tx of hemophilia A and B

A

Desmopressin (DDAVP)- Hemophilia A (mild)

Factor concentrates- Hemophilia A or B

75
Q

Von Willebrand dz

A 
The MC inherited bleeding disorder (autosomal dominant)
Type I (80%)- quantitative
76
Q

Clinical manifestations of von Willebrand dz

A

Mucocutaneous bleeding

Nosebleeds, easy bruising, bleeding gums

77
Q

Labs for vWB disease

A

vWF antigen measured functionally in the ristocetin cofactor assay

78
Q

Tx of vWB dz

A

Desmopressin
vWF-containing concentrate
Vaccine

79
Q

DIC

A

Abnormal coagulation and fibrinolysis
Usually associated with shock/sepsis
Coagulation factors are consumed as are anticoagulant proteins

80
Q

Lab findings of DIC

A

Decreased platelets and fibrinogen
Prolonged PT, aPTT
Elevated D-dimer

81
Q

Tx of DIC

A

Treat the disorder including DIC first

Support the pt (hypoxia, acidosis, factors, platelets, etc)

Peds (11 decks)

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