Severe Unresponsive Thrombocytopenia in a Transplantated Patient with Chronic Werlhof ‘s Disease: Correction after Splenectomy

Claudio Campieri, MD, Nephrology Department, S. Orsola University Hospital, Via Massarenti 9, 1-40138 Bologna (Italy) Dear Sir, Werlhof s disease is one of the commonest causes of platelet depletion [1, 2]; it can affect patients with a renal transplantation. We describe the case of a woman in whom this disease manifested itself with a severe clinical picture only 13 years after transplantation. This disease was being checked in its expression by the association of immuno-suppressive drugs (azathioprine and steroids) assisting the transplant, since they are also known to be effective as Werlhof s disease therapy. This treatment changed the expected clinical outcome in so far as it delayed the identification of the problem by limiting the degree of platelet destruction. Careful investigation of the case produced the clues to proper diagnosis and effective treatment. Case Report A 42-year-old woman with chronic renal failure secondary to glomerulonephritis underwent cadaver donor renal transplantation in January 1980. The graft functioned well, with 75 mg/day azathioprine and 5 mg/day prednisone. In December 1993, severe platelet depletion was detected (16,000 platelets/ mm3). An accurate revision of previous records revealed 3 occasional drops in the platelet count in the posttransplantation follow-up to values as low as 50,000-100,000 platelets/ mm3, followed by spontaneous normalization. Episodes of metrorrhagia had persisted even after cavity overhaul for polypectomy of the cervix uteri in June 1980. In April 1992, for the first time, platelets fell to 50,000/mm3, but the search for antiplatelet antibodies proved negative and the medullary biopsy showed normal maturing normal-cell marrow, with a megakaryocyte count in the normal range. Creatininemia was 1.5 mg/dl.