Review Question - QID 104622

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Nephrotic Syndrome Complications Minimal change disease QID: 104622 (M2.RL.17.80)

QID 104622 (Type "104622" in App Search)

A 23-year-old male presents with enlarged cervical nodes. The nodes are non-erythematous, non-tender, mobile, and have a rubbery consistency. He notes recurrent night sweats that soak through his clothes and nightly fevers. A lymph-node biopsy is performed, and is shown in Figure A. The patient also mentions that his urine is “frothy”. A urine dipstick is positive for albumin (3+). If a renal biopsy were to be performed, which of the following would most likely be found in this patient?

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Normal glomerulus on light microscopy

44%

4/9

Eosinophilic nodular glomerulosclerosis

0/9

Hypercellular glomeruli with many neutrophils

22%

2/9

Mesangial proliferation

11%

1/9

Red-green birefringence under polarized light on congo red stain

22%

2/9

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This patient’s symptoms and lymph node biopsy finding are consistent with Hodgkin lymphoma (HL). Minimal change disease (MCD) has an association with HL. On light microscopy, the glomeruli would appear normal; however, on electron microscopy, glomeruli will show effacement of the foot processes, which explains the patient's "frothy" urine and proteinuria.

HL is a hematologic malignancy of B-cells in the lymph nodes. Patients with HL classically present with painless, non-erythematous, rubbery-like enlarged lymph nodes. These enlarged nodes can be found in the cervical, axillary, or supraclavicular region. Patients can also present with B symptoms: fever, night sweats, and weight loss. Reed-Sternberg cells can be found on lymph node biopsy. MCD can be seen in children, as well as in adults. Though the etiology of MCD is unknown and its pathogenesis is not well understood, there seems to be an association of MCD and HL.

Kodner discusses the diagnosis and management of nephrotic syndrome. Patients can present with hypoalbuminemia, proteinuria, hyperlipidemia, and edema. About 25% of cases of primary nephrotic syndrome are due to MCD and IgA nephropathy. On renal biopsy microscopy, the glomeruli are normal-appearing in MCD, with electron microscopy showing effacement of the foot processes.

Audard et al., report 21 cases and review of the literature in relation to MCD and classical HL. MCD has been described as being a paraneoplastic manifestation of classical HL. A retrospective study was performed to evaluate this association. In some patients, MCD was the first presentation before classical HL was diagnosed, which seemed to be steroid resistant or dependent. Effective treatment of the classical HL had an association with MCD resolution.

Figure A shows a Reed Sternberg cell, which is CD15+ and CD30+. HL is characterized by the presence of these cells.

Incorrect Answers:
Answer 2: Eosinophillic nodular glomerulosclerosis describes the Kimmelstiel-Wilson lesion seen in diabetic nephropathy.
Answer 3: Hypercellular glomeruli with many neutrophils is suggestive of a post-infectious glomerulonephritis.
Answer 4: Mesangial proliferation is seen in IgA nephropathy (Berger disease). Immune complex deposition within the mesangium leads to the activation of mesangial cells, which subsequently causes mesangial hypercellularity.
Answer 5: Red-green birefringence under polarized light on congo stain describes renal amyloidosis.