For Goldstein Report, PGY3 Robert Tamai presented a case of a 52 y/o male with no PMH who was brought to the ED by his family for worsening confusion and generalized weakness x 2weeks. The family also noted more yellowing of his skin during this time. ROS significant for chills, sore throat, cough, anorexia and jaundice. Patient does not have any past surgical history of home medications. He drinks beers daily but no smoking or other drug use. Physical exam with stable vital signs, +scleral icterus, mildly jaundiced, 3cm palpable R anterior cervical lymph node and A&Ox1 (person only). DRE negative for blood.
CBC: WBC 8.3 > Hb 5.4/Hct 16.2 <Plt 140
Na 131/K 3.4/Cl 91/HCO3 18/BUN 14/Cr 0.99 <Glucose 155 (Anion Gap: 25.25 after correcting for albumin)
ALP 351>T.protein 6.2/Albumin 2.7/AST 108/ALT 49/Tbili 3/DBili 2.2
Coags: PT 21.4, INR 1.88, PTT 40.6
VBG: 7.42/26/39/18, lactate 4.2
Infectious workup (BCx, UA) negative. Hepatitis panel negative. HIV and RPR negative. Procal elevated at 1.9 ng/mL.
Altered mental workup including CTH, UTox, ETOH, ammonia, APAP, and TSH were all negative.
Iron panel: Iron 22, TIBC and Iron Sat unable to assess due to ferritin interference, Ferritin 5429
Workup of anemia and coagulopathy:
- Haptoglobin: 303 (normal: 30-200)
- LDH 212 (normal: 135-225)
- Ddimer 1.65 mcg/mL (normal <0.49)
- Fibrinogen 716 (normal: 228-487)
- Factor V: 80% (normal 70-120%)
- Factor X: 51% (normal 60-150%)
- DAT negative
- Peripheral smear: 1 defective nucleated RBC, 1 mononuclear cell otherwise normal RBC morphology. No toxic granules or hyposegmented neutrophils.
Learning point from Dr. Feinstein about the coagulopathy: A high fibrinogen over 700, rules out liver failure. Factor V was 80% (synthesized in liver and NOT vitamin K dependent) and vitamin K dependent Factor X was 50%. Thus, the patient had vitamin K deficiency accounted for the prolonged PT and PTT.
Abd US showed hepatosplenomegaly but no surface nodularity to suggest cirrhosis. CT A/P w/ contrast again showed hepatosplenomegaly, periportal and retroperitoneal lymphadenopathy. CT Multiphase liver showed hepatosplenomegaly and heterogeneity of the hepatic and splenic parenchyma due to an infiltrative process. Stable thoracic, retroperitoneal and mesenteric lymphadenopathy.
Bedside EGD was done initially given concern for possible GI bleed. Esophageal candidiasis found but no esophageal or gastric varices found.
PET scan with hypermetabolic LNs throughout, splenomegaly, multiple areas of focal FDG uptake in the liver (max SUV to 5.5):
Excisional R neck LN biopsy done which showed Classic Hodgkin lymphoma.
Treatment: Patient was started on steroids and chemotherapy with improvement of mental status to A&Ox3.
Take home points:
- Differential of infiltrative liver disease: sarcoidosis, TB or fungal infections, amyloidosis, malignancy (lymphoma, metastasis, HCC) or hepatic extramedullary hematopoiesis.
- Primary hepatic lymphoma (PHL) is extremely rare (only 100 case reports), characterized as lymphoma confined to liver and perihepatic lymph nodes and seen in patients who are chronically immunosuppressed. No BM involvement noted.
- Secondary hepatic lymphoma common indicated advanced disease (50% in non-Hodgkin lymphoma and 20% in Hodgkin lymphoma). There will be BM involvement, systemic lymphadenopathy and hepatosplenomegaly.
The imaging conundrum of hepatic lymphoma revisited