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Pale, Bleeding, and Febrile:Heme-Onc Emergencies in Kids

Judith R. Klein, MD, FACEPAssistant Professor of Emergency Medicine

UCSF-SFGH Department of Emergency Medicine

Case #1: Polka dot Jane

3 yo spots on her legs x 1 week, gum bleeding and epistaxis

Recent viral illness

PE: vs nl; bruises, petechiae, no splenomegaly

Labs: Plts 20K, other cell lines normal

Bleeding in kids

Let’s talk bleeding: -Deep (muscles/joints): factor prob -Mucocutaneous (gums, nose): platelet prob

Hx: meds, recent infxn, family hx

PE: VS, ill?, splenomegaly

Labs: CBC/smear, PT/PTT, fibrinogen, d-dimer, lytes

<150K platelets abnormal

What happened to the platelets?

DESTRUCTION PRODUCTION

ITPITPITP

Medications (heparin)HUSDIC

Bone marrow problem:

-infiltration -aplasia

Rare in kids

Immune thrombocytopenic purpura: ITP

Age 2-4 yrs; 80% resolve in 6 mos

Sudden bleeding/bruising post viral

Treatment: admit/heme consult!

>30K/mild bleeding: observe

<20K or significant bleeding: -IVIG, steroids (BM biopsy) -ICH or life-threatening hemorrhage? platelet transfusion/splenectomy

Case #2: Pale, bleeding and feverish

2 yo with pallor and low grade fever x 10 days. Blood on toothbrush x 1 month

PE: P120, BP 90/50, T 38.0 blood at gums, pale, diffuse LAN, spleen tip palpable

Labs: Hb 6, Plt 20, WBC 120K; blasts on smear

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Leukemia in the ED

Most common CA in kids: 75% ALL

Differential: -Virus (EBV/parvovirus) -Autoimmune (HUS) -BM failure (aplastic anemia)

Workup: smear, lytes, Ca, PO4, uric acid, T/S, PT/PTT, BCx/Abx if T>38.3, CXR, EKG

Hyperleukocytosis

WBC>100,000-->hyperviscosity -->sludging-->CNS/lungs most affected

Tx: oncology ASAP for leukopheresis and/or immediate chemo

Beware transfusions-->can worsen viscosity problem

More bad news.....

K+

PO4

Uric Acid

Ca 6.5, PO4 7, uric acid 10 and K 7.5!!

Tumor lysis syndrome--> arrhythmias/renal failure

Treatment: -Hydration -Hyperkalemia/hypocalcemia tx -Hyperuricemia *Alkalinization? Allopurinol? Rasburicase?

-Hemodialysis

Case #3: He looks like Shrek

15 yo no 1o care large LN in neck bigger x 2 weeks; face fatter; dry cough at night

PE: VS wnl, nl resp status, 5 cm neck LN firm, painless; facial plethora

Labs: mild anemia, nl lytes/ uric acid, CXR mediastinal mass/tracheal deviation

Isolated lymph node enlargement

#1: Lymphadenitis- try antibiotics, consider MRSA

Hx of cat scratch? TB? Monospot?

Recheck 1 week: bigger, firm, fluctuant, diffuse-->more aggressive work-up: US, CXR, CBC, FNA biopsy

Lymphoma in kids

#3 cause of CA in kids after leukemia and brain CA.

Hodgkins>>NHL

Very curable, but initial presentation may be life-threatening

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Mediastinal masses: disasters waiting to happen....

#1 problem: airway

SVC syndrome

Management: -AVOID SEDATION -Oncologist for further imaging (CT/MR) and emergent chemo/radiation/steroids

Case #4: Hot Tot

3 yo with AML with T101 x 2 days; rhinorrhea/mild cough; last chemo 5 days; Imm UTD

PE: T 100 P120 RR 30 O2 96%; mod mucositis, nl CRT, CVL site clean

Labs: WBC 1.8, ANC 350, PLT 40

Fever/neutropenia in kids

Definition: -T>101 or >100.4 x 2; oral or ax -ANC<500mm3

Many causes of neutropenia

Bugs involved: -10-30% ID’d: rest idiopathic -90% bacterial (skin, resp, GI) -Viral (HSV, VZV, RSV, flu): less common -Fungal: prolonged neutropenia/steroids

Admit them all?

Not all fever/neutropenia alike

Mortality: 80% to 1-3%

Risks of admitting: -nosocomial infection -kids/parents hate it -$$$

Risks of not admitting: -overwhelming sepsis

Risk profileHigher risk:

-<1 year old -Lower/longer neutropenia -Focal infection -Severe mucositis -VS abnl/shock/organ failure -Indwelling device: CVL -CRP>90, PLT<50

Lower risk: -T<39; no focal infection -no sx except fever

The Science?

Gupta, 2009: -123 episodes: 88 pts 2-15 years -Criteria: no focal infxn or sepsis, no other reasons for inpt, brief low ANC, no hx fungal infxn

Augmentin/oflox po vs. CTX/amikacin IV

No difference in fever resolution, no mortality

SMALL study

Gupta, Ped Hemat Oncol 2009.

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More science on fever and neutropenia

Agyeman: Predicting bacteremia

423 episodes fever/16% bacteremia

100% sensitivity if any one:

Hb>9, Plt<50, shaking chills, other other reason for admit

Derivation only; needs validation

Agyeman, Ped Infec Dis J 2011

Fever/neutropenia bottom line

Admit unless pediatric oncologist directs otherwise or patient in clinical trial

Fever/neutropenia: management

Workup:-All: CBC, BCx, UA,*CXR -Sx dependent: chem 7, nasal wash (rsv, flu), throat cx, skin swabs

Treatment: -Neutropenic precautions!-Abx for G+/G-/pseudomonas: ceftazidime, cefipime, imipenem, zosyn -Add vancomycin if CVL -G-CSF: no evidence of mortality benefit -Hydrocortisone: only if on steroids or pituitary abnl

Case #5: My bones ache

15 yo with SCD/asthma c/o leg, back, chest pain. Mild cough.

PE: T 38.2, RR 18, O2 96% chest: few wheezes; legs/back hypesthetic

Labs: Hb baseline, retics>10% CXR....

Pain crisis

Duration: 3-7 days

Most common complication of SCD -3x more admit 25-29 yrs vs. <4 yrs

Low back>long bones> abd/chest

PE: 20% fever, usually normal

Red flags: HA, CP, abd pain, jaundice, vomiting, neuro sx, focal bone ttp/edema, fever

Redding-Lallinger, Curr Prob Ped Adolesc Health Care 2006

Pain crisis management

PO or IVF to euvolemia

Pain meds: NSAIDs, opiates, PCA D/C: oral pain meds RTC not prn

Steroids?: NO, shorter episode but rebound pain common

Nitric oxide? No benefit in 150 pt RCT*

*Gladwin, JAMA 2011.

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What about that CXR?

Acute chest definition: new infiltrate + CP, T, O2, RR, cough, or wheezing

Pneumonia vs acute chest?

Pathophysiology/risks: -pain crisis (necrotic BM)-->fat embolism -sedation/splinting-->hypoventilation -asthma (RR 6-8)*

*Knight-Madden, Thorax 2005.

Acute chest: management

Pain management

Oxygen/incentive spirometry*

Abx: cephalosporin (CTX)+macrolide

Bronchodilators prn

pRBC: if deteriorating; to Hb 10 only

Future: NO? steroids?

*Bellet, NEJM 1995.

Case #9: Hot sickler

5 mo old SCD and T 39 x 2 days. No other sx; no sick contacts. Nl po and UOP. Immuniz-UTD; on PCN

PE: T 39.1, RR 30, O2 98% appears well, nl CRT, no resp distress. No bone ttp. No rash.

Bacterial infection and SCD

Most common cause of death

Pathophysiology: functional asplenia

Bugs: Pneumococcus, Salmonella, Staph, E. coli, Strep.

Highest risk: <12 mos up to 3 years

SCD: 300-600x risk of Pneumococcus (IPD)

PCN prophylaxis: reduce IPD by 84%

Impact of vaccinations

Vaccines: H.flu and PCV-13

McCavit 2011: 3x risk of hospitalizations post PCV-7

Adamkiewicz 2008: 68% risk of IPD post PCV-7.

McCavit, Ped Blood Ca 2011

Adamkiewicz, Pediatrics 2008

Work-up: fever and SCD

Fever: T>38 if <6 mo; >38.5 if >6 mo

Hx: Immuniz? Pcn? Focal sx?

Workup: CBC, retics, BCx; +/- : UA/UCx, CXR, LP

Abx: ceftriaxone for all; +/- vanco

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Fever/SCD: disposition?

Admit: -all <6 mos - >6 mos if toxic, other SCD complic, T>40, WBC <5 or >30, Hb<5

Discharge: -return 24 hrs for re-check -repeat abx until Cx (-) x 48 hours and afebrile -reliable pt

In a nutshell

Bruising and low platelets? Think ITP but admit them to rule out bad things

Not just platelets low? Bone marrow problem/cancer

Mediastinal mass? Beware of sedation

Admit febrile neutropenic kids. Period.

SCD and pain? May be just a pain crisis, but look at end organs

SCD and fever? It may not be a virus!