American College of Physicians Kansas Chapter Conference

October 3, 2013

Ky Stoltzfus, MDUniversity of Kansas Medical

Center

Have I got a case for you...

Or should it be:

Have I got a case for you?

62 year old man with acute promyelocytic leukemia

presents with shortness of breath and chest pain.

HPI: Chest pain over left sternum, dull,

7/10 severity, constant, began 4-5 hours prior, not relieved or worsened by any factors.

Associated SOB, started at same time, some cough and white sputum. Can't lay flat easily, gets “winded” with walking.

Recent diagnosis of APL Bone marrow hypercellular 95%

with 80% blast or promyelocytes Started All-Trans Retinoic Acid

(ATRA) therapy the day of admission

During visit he was noted to have WBC 0.7 K/uL Hgb 7.5 g/dL Platelets 13 K/uL

Transfused 1 unit platelets

ROS

Positive for the following, otherwise negative: Gen: fatigue, malaise, anorexia CV: chest pain Pulm: SOB, cough, sputum production Neuro: dizziness

PMH HTN CAD Type II DM Atrial fibrillation

PSH None

Meds tretinoin

• flecainide

• simvastatin

• zolpidem

• fish oil /omega-3 fatty acids

• atenolol

• polyethylene glycol (MIRALAX)

• pantoprazole

Soc Hx Married Nonsmoker, no EtOH, no illicit drugs

Fam Hx Father – prostate CA, died 82yo Mother – CAD, HTN, living 84yo Siblings – healthy No other cancer history

Physical Exam

38.1C P99 R21 BP110/78 O2 87%RA

Gen: Sitting, in moderate respiratory distress, alert, oriented x 3

Neck: No carotid bruits, no JVD

CV: Irregular, no S3 or S4, no murmur

Pulm: Crackles in bilateral bases and mid-lung fields

Abd: Soft, nontender, nondistended

Extrem: no cyanosis or edema

Pulses: 1+ bilateral radial, dorsalis pedal, posterior tibialis

EKG: atrial fibrillation, rate 99, LVH, no ST or T wave changes, no Q waves

Labs:

Hgb 7.5, WBC 0.8, Plat 27

32%N, 3%Band, 30L, 4M, 31% blasts

Na 131, Cl 101, bicarb 22, lactate 2.1, Cr 1.4, Tbili 1.5, LDH 299

Trop 0.01, BNP 185

What's in your differential diagnosis?

Here's mine: CHF exacerbation Transfusion Associated Cardiac Overload

(TACO) PNA, atypical TRALI (Transfusion Associated Acute Lung

Injury) PE

What would you do next?

Diurese patient Possible emperic antibiotics Consider CT chest or VQ scan Contact your blood bank

TRALI

American Society of Hematology Education Program

http://asheducationbook.hematologylibrary.org/content/2006/1/497.full

TRALI

TRALI is characterized by acute non-cardiogenic pulmonary edema and respiratory compromise in the setting of transfusion

Normal CVP and wedge pressure

Mimics ARDS

TRALI attributed to donor leukocyte antibodies.

Alternate mechanism: “two hit” or “neutrophil priming” hypothesis.

Incidence

1:432 whole blood platelets

1:557,000 red cells

Plasma transmission variable (depends on region of the country)

Testing

HLA class I or class II, or neutrophil-specific antibodies in donor plasma and the presence of the cognate (corresponding) antigen on recipient neutrophils.

Takes weeks to obtain this.

TRALI is still a clinical diagnosis.

Follow up

Extremely important to notify your blood bank if TRALI is suspected.

Donors can tracked.

FDA is notified.

Case continued

Patient had worsened respiratory failure and subsequent multi-organ failure. He died in ICU on maximal life support.

Summary

Suspect TRALI if respiratory symptoms follow transfusion.

Keep your differential diagnosis broad.

Report suspected cases of TRALI to blood bank immediately.