27 Nov 2020

8/31/20: Follicular Lymphoma

PGY3 Ashwini Mulgaonkar presented a case of a 59 year old female with history of dyslipidemia who presents with malaise, nausea/emesis and abdominal pain x 1 week.  2 weeks prior to admission, patient developed cough and chest congestion.  At that time, she was diagnosed with bronchitis and prescribed azithromycin and antitussive.  After completing the course of antibiotic, she started developing abdominal pain with progressive nausea and non-bloody, non-bilious emesis and was prescribed Ranitidine.  The medication did not improve her symptoms and she started to develop 2-3 days of worsening PO intake and presented to the ED.  Ancillary history is insignificant.  Denies ethanol/tobacco/drug use.

Vital sign stable.  Physical exam with normal cardiovascular exam, lung with reduced breath sounds in bilateral bibasilar lung fields, mildly tender to palpation in epigastrium and palpable spleen tip.

CBC: WBC 6.9 > Hb 11.9/Hct 35.9 <Plt 160

CMP:

Na 136/K 5.2/Cl 94/HCO3 14/BUN 90/Cr 13.32 <Glucose 92

ALP 127>T.protein 7/Albumin 4.5/AST 21/ALT 9/Tbili 0.4/Dbili <0.2

Coags:PT 14.9, INR 1.19

Lipase 83 U/L (normal: 7-60)

Infectious workup:

  • Bcx negative x2
  • UA with 100 protein, small leuk (4-10 WBC), neg nitrite, Large blood (RBC >50)
  • UCx negative
  • Lactate 0.7 mmol/L
  • Hep A Ab IgM neg
  • Hep B Core Ab Nonreactive
  • Hep B Core IgM Nonreactive
  • Hep Bs Ag Nonreactive
  • Hep C Ab Nonreactive
  • HIV Nonreactive
  • RPR Nonreactive
  • Anti-Streptolysin negative
  • Anti-GBM negative

Renal workup:

  • Uosm 245 mOsm/kg
  • FeNa 16.4%
  • FeUrea 55.9%
  • Urine protein/cr 1.67
  • HbA1c 6%

Autoimmune workup:

  • ANA negative
  • ANCA negative
  • C3 135 mg/dL (normal: 90-180)
  • C4 19.8 (normal: 10-40)
  • dsDNA Ab Positive, 1:20
  • PLA2R Ab negative

Hematology workup:

  • Reticulocyte percentage 0.96%
  • Abs Retic: 36.38 x10^9/L
  • Iron panel
    • Iron 50 mcg/dL (normal 50-160)
    • TIBC 242 mcg/dL (normal 250-430)
    • Iron sat 21% (normal 15-50%)
    • Ferritin 249 ng/mL (normal 15-240)
  • LDH 213 U/L (normal 135-225)
  • Uric acid 18.9 mg/dL (normal: 2.7-6.4)

Renal US showed bilateral echogenic kidneys, consistent with renal parenchymal disease.  Mild bilateral hydronephrosis, L>R.

CT Chest/Abdomen/Pelvis revealed multiple enlarged lymph nodes above the diaphragm, bilateral pleural effusions, bulky confluent retroperitoneal mass-like soft tissue surrounding the aorta and IVC and extending inferiorly into the pelvis bilaterally with very bulky disease, splenomegaly at 15.2cm, mild bilateral hydronephrosis, secondary to proximal ureteral obstruction by the bulky retroperitoneal soft tissue.

PET scan showed extensive, diffuse nodal disease involving all nodal stations above and below the diaphragm, involvement of the spleen and bone marrow as well.

R axillary core lymph node biopsy revealed final diagnosis of Follicular Lymphoma, Grade 1-2 of 3.

Treatment: Patient was started on HD upon admission due to renal failure.  Hematology consulted after findings from CT which were suspicious for lymphoma.  Urology placed bilateral ureteral stents for obstruction.  She was transferred to Hematology wards for initial of steroids x5 days after LN biopsy resulted in follicular lymphoma.  She received Cytoxan x1 dose for debulking and was discharged without need for further HD.

Take home points:

  1. The degree of hydronephrosis seen on US correlates poorly clinically, especially for upstream obstructions.  The best test is NM renal differential scan with Lasix.
  2. Follicular lymphoma is the second most common type of non-Hodgkin lymphoma, characterized by the presence of a t(14;18) translocation that causes an overexpression of the BCL2 oncogene.
  3. Clinical presentation includes painless peripheral adenopathy in all regions that can commonly wax and wane spontaneously, asymptomatic large abdominal masses with or without evidence of GI and/or urinary tract obstruction and 20% will present with B symptoms.
  4. Grading: 1 (0-5 centroblasts/hpf), 2 (6-15 centroblasts/hpf), 3 (more than 15 centroblasts/hpf)
  5. For Grade 1-2, stage with imaging (CT or PET) to determine treatment course and for grade 3, if there is aggressive clinical presentation, will need to start anthracycline based chemo regimen (eg. RCHOP).

Review Article:
Follicular Lymphoma: 2020 update on diagnosis and management

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