MKSAP: 42-year-old woman with lymphadenopathy, fatigue, and edema

MKSAP: 42 year old woman with lymphadenopathy, fatigue, and edemaTest your medicine knowledge with the MKSAP challenge, in partnership with the American College of Physicians.

A 42-year-old woman is evaluated for a 3-month history of progressive cervical lymphadenopathy, fatigue, night sweats, bilateral lower-extremity and abdominal wall edema, and a 4.5-kg (10.0-lb) weight gain. History is significant for three episodes of weight gain and facial and lower-extremity edema lasting 4 weeks in her 20s and 30s. Her only current medication is a multivitamin.

After an evaluation and lymph node biopsy, she is diagnosed with stage IIIB Hodgkin lymphoma.

Laboratory studies:

Serum creatinine 1.3 mg/dL (114.9 µmol/L)
Urinalysis 2+ blood; 4+ protein; dysmorphic erythrocytes and occasional granular casts
Urine protein-creatinine ratio 9.25 mg/mg

On kidney ultrasound, the kidneys are 13.5 cm bilaterally and edematous. The corticomedullary junction is apparent, and there is no hydronephrosis.

Which of the following is the most likely cause of this patient’s nephrotic syndrome?

A) Focal segmental glomerulosclerosis
B) IgA nephropathy
C) Membranous glomerular nephropathy
D) Minimal change disease

MKSAP Answer and Critique

The correct answer is D) Minimal change disease. This item is available to MKSAP 15 subscribers as item 15 in the Nephrology section. More information about MKSAP 15 is available online.

This patient has minimal change disease secondary to Hodgkin lymphoma. Minimal change disease is a relapsing-remitting condition that may occur secondary to NSAID or lithium use, mononucleosis, or malignancy and may be the presenting symptom of Hodgkin lymphoma. Minimal change disease is characterized by sudden, massive proteinuria associated with a urine protein-creatinine ratio that may exceed 9 mg/mg. This condition also may cause mildly elevated blood pressure, hypoalbuminemia, and anasarca.

Proteinuria that develops after remission of Hodgkin lymphoma often indicates disease relapse. Therefore, close monitoring of the protein-creatinine ratio is indicated once remission is achieved to evaluate whether additional therapy is needed after chemotherapy and radiation therapy are completed.

In patients with solid tumors, the nephrotic syndrome is usually associated with membranous glomerular nephropathy; however, minimal change disease predominates in those with hematologic malignancies, particularly Hodgkin lymphoma.

Focal segmental glomerulosclerosis is the least frequently reported cause of the nephrotic syndrome in patients with hematologic malignancies and most often develops after transplantation or intense chemotherapy.

IgA nephropathy is not commonly associated with lymphoma or myeloproliferative disorders.

Key Point

  • Minimal change disease may be the presenting symptom of Hodgkin lymphoma and is characterized by sudden, massive proteinuria.

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