22 Oct 2020

10/5/20: Burkitt Lymphoma

PGY3 Swathi Nallapa presented a case of a 51 y/o male with no known PMH presents to ED two weeks after a syncopal episode.  The patient reports he was on vacation in Paris and was attempting to stand up quickly and suddenly passed out and woke up 15 mins later.  Denies any shaking, tongue biting or bowel/bladder incontinence.  Patient denies any prodromal symptoms of nausea, flushing, lightheadedness, chest pain, SOB or palpitations prior.  Further history revealed that the patient has had a recent febrile illness approximately 1 month ago with an associated dry cough.  Has noted 2 weeks of dyspnea on exertion with increasing fatigue and b/l LE edema (R >L) x 1 week.  For social history, patient works in TV production and travels frequently.  He is a married to a male partner x3 years.  Has a 40 pack year smoking history, last smoked 2015.  Has drank alcohol since age 18, drinking 4-5 beers daily and additional binge drinking on weekends.  Denies an IVDU.

Vitals: T 37.5, HR 122, RR 20, BP 110/69, O2 sat 93% 2L NC

Physical Exam significant for tachycardia, bibasilar crackles up to the mid lung fields, and 1+ pitting edema up to the knees.

CBC: WBC 7.1> Hb 11.1/Hct 33.7 <Plt 332 (MCV 88, RDW 14.5%)

CMP:

Na 131/K 5.1/Cl 96/HCO3 16/BUN 26/Cr 0.86 <Glucose 86

ALP 115>T.protein 7.6/Albumin 2.3/AST 216/ALT 164/Tbili 0.6/Dbili 0.2

Coags: PT 17.5, INR 1.45, PTT 33.7

Cardiac workup with elevated BNP and TTE showing normal EF but moderate pericardial effusion seen.

  • EKG:

CXR

CTPA shows likely lymphoma with involvement of mediastinal fat, epicardial fat and pericardium. Small filling defect with linear configuration in the LUL pulmonary artery lobar branch.  Pleural effusions and scattered mass-like areas of consolidation.

Infectious workup significant for HIV positivity with a viral load of 864,940 and CD4 count of 426.

Heme workup showed elevated LDH at 1136 (normal <125) and elevated Ferritin at 1389 (normal <330).

PET shows hypermetabolic masses involving the anterior mediastinum and invading into the pericardium and duodenum; hypermetabolic retroperitoneal and porta hepatis nodal basins, spleen and bone marrow are suspicious for underlying lymphoma.

CT guided biopsy of the anterior mediastinal mass done, showing Burkitt lymphoma.

Treatment: Patient was transferred to Heme wards for initiation of chemotherapy.  Tumor board held and decision made to start R-EPOCH minus anthracycline due to the RV dysfunction with plan to receive R-CODOX-M next cycle.

Teaching points:

  1. AID defining malignancies: Kaposi Sarcoma, Non-hodgkin lymphomas (systemic high grade B cell lymphomas and primary CNS lymphoma)
  2. Burkitt lymphoma (BL) typically affects those with a relatively high CD4 count (>200) and no opportunistic infections.  Incidence has NOT decreased with advent of ART.
  3. Chronic EBV infection is known to play a role in almost all cases of endemic BL.
  4. Patients with BL typically present with rapidly growing tumor masses and often have spontaneous tumor lysis with high LDH and elevated uric acid.

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