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Heme/Lymph II Exam II > Leukocytosis and Leukemias in pediatric pt (Brandau) > Flashcards

Leukocytosis and Leukemias in pediatric pt (Brandau) Flashcards

1
Q

There is prominent periorbital
Swelling and a vesiculopustular
rash on the face and trunk

PE unremarkable

no LAD

splenic tip palpable
liver edge descended 5 cm below costal margin

WBC–> 73,200 (very high)

52% blasts
atypical lymphocytes

platelets 973,000 high!

lactate dehydrogenase high (lots of cell turnover)

CSF - blasts

A

Transient myeloproliferative disorder with trisomy 21 mosaicism

some cells have trisomy 21

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2
Q

tzanck test?

A

herpes infection

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3
Q

in herpetic infections what is going on with the WBC count and predominantly what cell type?

A

WBC count tends to be marginally elevated and predominated by lymphocytes later in the course of the illness

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4
Q

what is leukemoid reaction

A

defined as the elevation of normal leukocytes to counts greater than 50,000 cells/μL

must be distinguished from CML

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5
Q

decreased margination of leukoctyes

A

Exercise

Catecholamines

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6
Q

neutrophilic leukoctyosis

A

Acute bacterial infections, especially those caused by pyogenic organism; sterile inflammation caused by, for example, tissue necrosis (myocardial infarction, burns)

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7
Q

eosinophilic leukocytosis

A

Allergic disorders such as asthma, hay fever;

certain skin diseases (e.g., pemphigus, dermatitis herpetiformis);

parasitic infestations; drug reactions; certain malignancies (e.g., Hodgkin and some non- Hodgkin lymphomas); collagen vascular disorders and some vasculitides; atheroembolic disease (transient)

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8
Q

basophilic leukocytosis

A

rare

Myleoproliferative disorder

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9
Q

monocytosis

A

Chronic infections (e.g., tuberculosis), bacterial endocarditis, rickettsiosis, and malaria; collagen vascular diseases (e.g., systemic lupus erythematosus); inflammatory bowel diseases (e.g., ulcerative colitis)

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10
Q

lymphocytosis

A

Accompanies monocytosis in many disorders associated with chronic immunological stimulation (e.g., tuberculosis, brucellosis); viral infections (e.g., hepatitis A, cytomegalovirus, Epstein-Barr virus); Bordetella pertussis infection

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11
Q

high LDH?

A

The elevated lactate dehydrogenase level also suggests the rapid proliferation of cells as seen in a neoplastic disorder

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12
Q

leukemic hiatus?

A

The peripheral smear did not show a morphological progression from blasts to mature forms. This observation is called the leukemic hiatus and suggests the blasts are autonomous suggesting a neoplastic process rather than an response to inflammation which would show the progression of elements of the granulocytic series

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13
Q

infant myeloid leukemia is associated with propensity to extramedullary disease in the what

A

skin and CNS

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14
Q

extremely high platelets are consistent with what

A

transient myeloproliferative disorder

Transient myeloproliferative disorder originally thought to only occur in patients with trisomy 21

Clinically appears identical to acute myeloid or acute megakaryocytic leukemia

The only distinguishing factor is the spontaneous resolution of all the hematological abnormalities, usually by 12 weeks of age

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15
Q

The skins rashes of true infant leukemia have been described as only nodular

A

true leukemia–> accumulation of leukemic cells in the skin!

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16
Q

CD34

A

stem cell associated antigen

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17
Q

CD45

A

pan-leukocyte antigen

18
Q

CD41

A

megakaryocyte associated antigen

19
Q

CD33

A

a myeloid antigen

20
Q

CD117

A

a early myeloid antigen

21
Q

median age of diagnosis of transient myeloproliferative disorder

A

7days

22
Q

A 3 y/o boy presents to the clinic with pallor, bruising and intermittent fever for the past 3 weeks.
Physical exam reveals no cause for his fever or pallor.

hemoglobin 6.8 low
WBC 1800 low
2% neuts 98% lymphocytes

bone marrow aspirate–> myeloblasts

A

leukemia!

the initial presentation was pancytopenia that mimicked aplastic anemia

50% of children with ALL and 20-30% with AML present with WBC

23
Q

A 13 y/o girl presented with malaise and intermittent fever for 1 week. She had bilateral 2-3 cm nontender anterior cervical nodes and splenomegaly. EBV titers were equivocally high. A presumptive diagnosis of mononucleosis was made and laboratory studies were drawn.

hemoglobin 10.5 low
WBC 135,000 high
platelets 40,000 low
LDH 4500 high
Uric acid 11 mg very high (cell turnover)

24hrs later
WBC differential- 95% blasts

A

lymphoblastic leukemia

Flow cytometry is positive for CD2 and
CD7 and identifies the T-cell origin of this acute lymphoblastic leukemia subtype

Case 2 is typical of high risk ALL
High Risk ALL presents with rapid onset, high tumor burden due to marked leukocytosis with lymphadenopathy and/or hepatosplenomegaly and mediastinal disease

About 20 % of patients with WBC > 50,000 will have this presentation

Sometimes the presentation is thought to be mononucleosis

24
Q

A 5 y/o girl presents with intermittent bilateral leg and wrist pain and fever as high as 103° F of 3 weeks duration. Physical exam is normal with the exception of the limp.

hemoglobin 10.9 low
MCV 77
WBC 4100
Diff- 21% neuts
79% mature lymphocytes 
platelets 190,000 
ESR 35 (elevated sed rate)

A diagnosis of juvenile rheumatoid arthritis is made and she is started on aspirin therapy. There is improvement over the next 2 days but then the pain worsens and she refuses to bear weight. A 2 week trial of naproxen provided no relief, Images of the wrists and legs were normal. Further testing was done.

LDH- 1200
uric acid 6.9
bone marrow aspirate -pre b cell lymphoblasts

A

Leukemia

The presentation with bone pain and fever occurs more commonly in the younger child
Fever is secondary to cytokine release from lymphoblasts but could also indicate a life threatening infection due to neutropenia
The bone pain is secondary to marrow expansion
These patients will frequently have normal range WBC
Elevated LDH and uric acid levels suggest a malignant process

25
Q

most common pediatric cancer

A

ALL -acute lymphoblastic leukemia

accounting for 25% of all cancers in children < 15 years of age

26
Q

peak incidence of ALL

A

2-5 yrs of age

more common in males

27
Q

what are the genetic syndromes associated with leukemias

A

Down syndrome-15-20 fold increase
Fanconi syndrome
Klinefelter syndrome
Shwachman-Diamond syndrome

28
Q

WBC greater than 200,000 run the risk of what

A

microemboli to brain or lungs suggesting a stroke or PE

more commonly seen in AML

29
Q

Respiratory distress, cough and facial edema suggest what?

A

superior vena cava syndrome due to mediastinal compresion of the airway or major vessels in ALL

30
Q

how does AML sometimes present that is life threatening

A

DIC

31
Q

what is in the differential diagnosis for leukemia non malignant conditiions

A 
Aplastic anemia
 	Juvenile rheumatoid arthritis
	 Viral infection
		—Infectious mononucleosis
		—Cytomegalovirus
	 Autoimmune thrombocytopenic purpura
	 Autoimmune pancytopenia/Evan syndrome
	 Sepsis
 	Leukemoid reaction
		—Pertussis
		—Acute infectious lymphocytosis
	Langerhans cell histiocytosis
	 Osteomyelitis
	 Hypersplenism
	 Megaloblastic anemia
32
Q

what is in the differential diagnosis of leukemia (malignancies)

A

Neuroblastoma
Retinoblastoma
Rhabdomyosarcoma

33
Q

how is the diagnosis of leukemia made definitively in a child

A

not JUST peripheral smear and WBC

bone marrow samples for morphology

flow cytometry and cytogenetics are necessary for diagnosis and to determine appropriate therapy

CSF is obtained to verify CNS leukemia is not present-this study must be done under optimal conditions (sedation, ultrasound guidance) to prevent a traumatic tap that introduce leukemic cells into the CNS

34
Q

what is the survival rate of children with ALL and AML

A

80-90% of children with ALL and 50% with AML are cured

35
Q

what are the lower risk factors for leukemia

A

age 1-9

low to normal WBC at diagnosis

hyperdiploidy (>50 chromosomes/cell)

36
Q

higher risk factors for leukemia

A

age>10,

infants with high WBC and

those with mature B cell leukemia

hypodiploidy

t9;22

37
Q

following induction of ALL, therapy for ALL lasts how long..

when are CNS prophylactics given ?

A

2-3 years

2 for girls
3 for boys

therapy in the first 6-12 months are more aggressive

Patients with bad prognostic indicators receive more aggressive intensified therapy

CNS prophylactics provided in all treatment regiments-mostly intrathecal chemotherapy

38
Q

general therapeutic concepts for AML

A

dramatically different than ALL

extremely intensive chemo for about 1 year

if available Bone marrow transplant from antigen identical sibling (found only 20% of time) is optimal

39
Q

what are the morbidities of leukemia treatment

A

infection!!!

management of fever is critical

Constant immunosuppressive therapy depresses lymphocyte function during therapy and for 3-12 months after therapy is completed

Live virus vaccines are CONTRINDICATED

40
Q

what are the tumor lysis syndrome characteristics?

uric acid
renal function
potassium
phosphate

A

● Release of intracellular uric acid, potassium, and phosphate from rapid
turnover of malignant cells

● Usually precipitated by chemotherapy, but can occur before
● Most often with high tumor burden or T-cell leukemia

● Components of tumor lysis:
—Hyperuricemia
—Renal precipitation can progress to acute renal failure
—Hyperkalemia
—Can progress to fatal arrhythmia
—Hyperphosphatemia/Hypocalcemia
—Increased phosphate can cause hypocalcemia and renal precipitation can
progress to renal failure
41
Q

how do you manage tumor lysis syndromes

A

Hydration and diuresis!!

supplemental potassium

treat hyperkalemia emergently if necessary

decrease uric acid with allopurinol or urate oxidase

consider oral phosphate binders

initiate dialysis for acute renal failure

transfer urgently to a pediatric oncology tertiary care center

Heme/Lymph II Exam II (5 decks)

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