Morning Report is back after taking a few weeks off! Here’s Dr. Waldman with today’s edition!
BLAST CRISIS: Medicine say what?
Case: 33 yo M irritable, yelling about SOB, abdominal pain, CP. After doctor approaches bedside, pt states he is not himself over the last several months he has persistent nausea, cannot tolerate food, SOB at rest, night sweats, body pain, and “lumps on his neck” which he is unable to give a clear history on time of onset.
Labs:
101.8> 6.3/21 < 41
Diff: 1% N 1 Band 30% Lymph 1% Mono 63% Blasts
Definition:
●≥20 percent peripheral blood or bone marrow blasts
●Large foci or clusters of blasts on the bone marrow biopsy
●Presence of extramedullary blastic infiltrates (eg, myeloid sarcoma also known as granulocytic sarcoma or chloroma)
PROMPT TREATMENT IS INDICATED BECAUSE, IF LEFT UNTREATED, THE ONE WEEK MORTALITY RATE IS APPROXIMATELY 20-40 PERCENT.
Symptoms:
- Pulmonary: dyspnea, hypoxia, diffuse interstitial or alveolar infiltrates on imaging, use pulse ox (arterial p02 can be falsely decreased bc of hyermetabolic WBCs)
- Neurological: visual changes, headache, dizziness, tinnitus, gait instability, confusion, somnolence, coma, increased risk for ICH (reperfusion injury)
- Fever- inflammation (cytokines) vs infection
- Less common signs: MI, AKI, priapism, acute limb ischemia, bowel infarction
Management: THERE IS LITTLE EVIDENCE TO CONFIRM THAT DECREASING THE WBC COUNT ALONE WILL REDUCE EARLY MORTALITY RATE. IN ADDITION, CLINICAL DETERIORATION MAY OCCUR EVEN AFTER THE WBC COUNT HAS BEEN SIGNIFICANTLY REDUCED.
- First Line: Induction chemotherapy and prophylaxis for TLS (allopurinol, acetazolamide, rasburicase)
- No symptoms, delay in induction chemotherapy: cytoreduction with hydroxyurea + IVF+ TLS prophylaxis
- Symptoms, delay in induction chemotherapy: cytoreduction with leukophoresis + hydroxyruea
“GIVEN THE PAUCITY OF DATA CONCERNING THE EFFICACY OF LEUKAPHARESIS IN REDUCING EARLY MORTALITY AND/OR IMPROVING OVERALL SURVIVAL, LEUKOPHORESIS CANNOT BE RECOMMENDED FOR ROUTINE THERAPY AS A FORM OF TUMOR “DEBULKING” IN PATIENTS WITH HIGHER BLAST COUNTS.”
Negatives for Leukophoresis:
- Thrombocytopenia worsened
- May require multiple sessions and may fail to lower WBCs even with multiple sessions
- Effect is transient
- Unclear if it can reverse vascular damage already sustained
- Symptomatic leukostasis can still develop after WBC count is lowered
Jay Khadpe MD
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