27 Nov 2020

11/5/20: Primary Effusion Lymphoma

PGY3 Hannah Gwin presented a case of a 40 year old homeless Hispanic male with history of meth use and HIV/AIDS c/b cryptococcal meningitis, cryptococcal choroiditis OD and CMV retinitis OS who presents with progressively worsening HA approximately 20 days after leaving OSH against medical advice wherein he received 3/10 days of Amphotericin and Flucytosine.  HA located throughout entire head and described as intermittent, pulsatile, severity 5/10 and associated with photophobia, eye pain and neck pain.  The patient also stated new intermittent chest/epigastric pain that is positional and worse with lying down; the patient attributes this discomfort to gastric reflux.  Patient reports compliance to home medications but unable to name specific meds.  He was born in Mexico and is currently homeless, living in a car.  Uses tobacco and crystal meth bue denies IVDU.

Vitals: T 37.0, HR 130, RR 18 BP 95/59, O2 sat 100% on RA

Physical Exam significant for A&Ox4, no focal deficits, palpable R inguinal LAD, multiple vesicular lesions on L side of lower lip, tachycardic with distended neck veins noted with JVD, positive hepatojugular reflex but no murmur/rubs/gallop, normal pulm exam and GU with 1cm nontender, raised lesion on L glans.

CBC: WBC 8.2> Hb 11/Hct 31.7 <Plt 137 (MCV 84, RDW 15.8%)

CMP:

Na 137/K 4.2/Cl 103/HCO3 26/BUN 15/Cr 1.13 <Glucose 102

ALP 87>T.protein 6.8/Albumin 3.6/AST 19/ALT 11/Tbili 0.6

Ca 8.1, Mg 1.8, Phos 2.6

Coags: PT 14.6, INR 1.2

Infectious workup started with the following significant results:

  • CD4 10
  • HIV viral load >70,000
  • GC/CT negative, RPR negative
  • Cryptococcus Serum Ag positive, >1:2560
  • CSF Crypto Ag positive, 1:80

Given the chest pain and physical exam findings concerning for pericardial effusion, a stat TTE was performed:

TTE showed large pericardial effusion with concern of tamponade.

Pericardiocentesis performed with the following results:

  • Volume: 800cc
  • Color: Red
  • Clarity: Moderate bloody
  • Nucleated cell count: 1485/cumm (PMN 16%, Lymph 10%, Mono/Histo 4%, Others 70%)
  • Glucose: 75 mg/dL
  • Protein: 5.3 g/dL (Serum Protein: 5.7 g/dL)
  • LDH: 2564 U/L (Serum LDH: 128 U/L)
  • ADA: 75.8 U/L (normal <9.2)
  • Micro: no organisms, MTB PCR negative, AFB Cx negative, Fungal Cx negative
  • Fluid cytology: Primary Effusion Lymphoma (HHV8 positive)

Treatment: Patient was started on amphotericin + flucytosine given known cryptococcal meningitis.  He was transferred to the CCU after discovering the large pericardial effusion and a pericardial drain placed.  He was transferred to Heme wards for initiation of chemotherapy for Primary Effusion Lymphoma with EPOC with Doxel.  Biktarvy was also started with close monitoring for IRIS.

Teaching points:

  1. Primary effusion lymphoma (PEL) is a B cell non-Hodgkin lymphoma (NHL) caused by HHV-8 and accounts for 4% of HIV-associated NHL.
  2. Clinical presentation: malignant effusions in pleural space, peritoneal cavity and pericardium.
  3. Treatment: chemotherapy + antiretroviral therapy (ART). ART given by itself can lead to clinical remission and thus ART should be started in HIV-positive patients with PEL.

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