Anasarca, and Lymphadenopathy in a Kidney Transplant Patient: A Diagnostic and Therapeutic Challenge.

Case Report

A 57-year-old male kidney transplant recipient, originating from Congo and living in Switzerland for 10 years, was referred to our emergency department on the 26th of March 2021 for dyspnea. The clinical examination revealed anasarca progressing over 2 months, bilateral lower limbs edema and hydrocele. There were no skin or mucosal lesions at presentation. Symptoms started shortly after the patient returned from a 2-week trip to Kinshasa, Congo. His past medical history was relevant for a living-donor kidney transplantation in March 2019, in the context of end stage renal disease due to diabetic and hypertensive nephropathy. The patient had a history of subclinical C4d positive acute antibody mediated rejection (ABMR), treated with 1 dose of Rituximab in October 2019. Comorbid conditions included insulin-dependent type 2 diabetes mellitus, hypertension, treated obstructive sleep apnea, and stable monoclonal gammapathy (MGUS) with IgG lambda (62.3 mg/L). Immunosuppression at admission consisted of Ciclosporin, Mycophenolate mofetil, and Prednisone 5 mg/day.

Diagnostic work-up prior to hospital admission included an ultrasound of the lower limbs excluding thrombosis, normal transthoracic echocardiography as well as blank urinalysis without proteinuria. A CT scan was performed on 10 March 2021 (Figures 1A,B) and showed bilateral pleural effusion, predominantly on the right side with passive contact atelectasis. There were no ground glass opacities.

FIGURE 1

Chest CT Scan, (A) Thoracic high, (B) Thoracic low.

In the emergency department, initial blood work-up showed normal renal function, with normal electrolytes. Serum albumin was normal. The blood count showed mild thrombocytosis and mild hypochromic microcytic anemia with a Hb of 122 g/L. Leucocyte count was 5.1 g/L, with mild eosinophilia (1.16 g/L), and lymphopenia (0.3 g/L). CRP was mildly elevated at 17 mg/L. EBV and CMV viremias were negative. Quantiferon tuberculosis (TB) test was negative.

A right thoracentesis of 3 L was performed, relieving the dyspnea. Pleural fluid was bloody (1.45 × 10^7/L erythrocytes) and filled criteria for an exudate. Pleural culture, PCR for TB, adenosine deaminase as well as cytology were all negative in the pleural fluid analysis.

Bronchoscopy with broncho-alveolar lavage was obtained and showed a cell count of 10^7/L, with 83% macrophages, 16% lymphocytes, and 1% neutrophiles.

A whole-body 18-FDG PET-CT was obtained (Figures 2A,B), and showed pathological diffuse peritoneal hypermetabolism, as well as hypermetabolic right inguinal and left axillary lymph nodes.

FIGURE 2

PET-CT, (A) axillary, (B) inguinal.

Test Questions

(1) The blood lymphocyte subsets show: CD3+ = 1,050/ml; CD4+ = 550/ml; CD8+ = 500/ml; CD19+ = 4/ml; CD56+ CD16+ (NK cells) = 124/ml. These results are compatible with:

(a) CD8+ cells depletion

(b) CD4+ cells depletion

(c) Severe B lymphocyte (CD19+) cells depletion

(d) Abnormal NK cells level

(e) Post transplant lymphoproliferative disease (PTLD)

(2) The broncho-alveolar lavage showed a cell count of 10^7/L, with 83% macrophages, 16% lymphocytes, and 1% polyneutrophiles. These results:

(a) Are compatible with community acquired pneumonia

(b) Are compatible with Mycobacterium tuberculosis infection

(c) Are compatible with SARS-CoV-2 infection

(d) Are compatible with intra-alveolar hemorrhage

(e) Are normal

(3) What procedure would you recommend as the next step towards diagnosis?

(a) Kidney graft biopsy

(b) Left axillary lymph node biopsy

(c) Abdominal surgical exploratory laparotomy

(d) Bone marrow biopsy

(e) Presumptive antituberculous treatment

(4) In terms of diagnosis, which answer is correct in this case?

(a) Because of his African origin, the patient is at increased risk for Kaposi sarcoma (KS)

(b) KS is secondary to HPV infection

(c) PTLD can be excluded because EBV viremia is negative.

(d) TB is excluded because of the negative TB Quantiferon test

(e) CMV infection is a possible diagnosis

(5) How would you manage immunosuppression in the case of suspicion of malignancy or disseminated infection?

(a) Stop Mycophenolate mofetil, keep Prednisone and ciclosporin

(b) Increase immunosuppression by increasing the ciclosporin trough level

(c) Stop all immunosuppression

(d) Increase immunosuppression by doubling the dose of MMF

(e) Increase immunosuppression by switching from ciclosporin to tacrolimus