Clinical and pathological characteristics of Castleman disease: an observational study in a Spanish tertiary hospital.

Castleman disease (CD) represents a heterogeneous group of lymphoproliferative disorders that share well-defined histopathological features. An observational study of patients with CD was conducted. A total of 53 patients had CD: 20 had the unicentric form (UCD) and 33 the multicentric (MCD) variant; 10 of the latter cases were infected with human herpesvirus-8 (HHV-8) and 23 were idiopathic (iMCD). Median age differed between UCD and iMCD (30 vs. 49 years, p = .004). Males were completely predominant in HHV-8-associated MCD (100%), and females were more frequent in UCD (75 vs. 48%, p = .06). Relapses were more frequent in iMCD (57 vs. 10% UCD, p = .002), and mortality was significantly higher in iMCD and the HHV-8-associated form with respect to UCD. We conclude that UCD is a benign disorder of younger ages and female predominance, while iMCD represents a different entity with more disease relapses and higher mortality.

Donor-derived multiorgan transmission of mixed P. malariae and P. ovale infection: Impact of globalization on post-transplant infections.

A 57-year-old man was admitted with fever and thrombocytopenia 1 month after renal transplantation. He had never received a blood transfusion or travelled outside Spain. A peripheral blood smear revealed Plasmodium malariae and P. ovale parasites, diagnosis confirmed later by malaria PCR. The donor, from Equatorial Guinea, had negative thick and thin blood smears and rapid malaria antigen test prior to organ donation. Peripheral blood malaria PCR was not performed during donor screening. The second renal recipient and the liver recipient were evaluated and were found to be asymptomatic. Thick and thin films and rapid malaria diagnostic tests were negative for both patients and blood for malaria PCR was sent to the referral laboratory. The index patient was treated with oral chloroquine diphosphate, with a favorable outcome and was considered cured. Malaria PCR was negative for the other renal recipient and positive for P. malariae and P. ovale curtisi for the liver transplant patient. Both were treated with oral chloroquine and the liver recipient also completed treatment with primaquine phosphate. This reported case of multiorgan transmission of mixed malaria infection highlights the importance of PCR-based tests for Plasmodium in the screening of donors from endemic areas.

Recommendations for the management of the haematological and onco-haematological aspects of Gaucher disease.

Current knowledge of the haematological and onco-haematological complications of type 1 Gaucher disease has been reviewed with the aim of identifying best clinical practice for treatment and disease management. It was concluded that: (i) Awareness of typical patterns of cytopenia can help clinicians distinguish haematological co-morbidities. (ii) Red blood cell studies and complete iron metabolism evaluation at baseline are recommended. (iii) Haemoglobin levels defining anaemia should be raised and used in Gaucher disease treatment and monitoring. (iv) Surgeons should be aware of potential bleeding complications during surgery in Gaucher patients. The higher incidence of multiple myeloma in Gaucher disease suggests that Gaucher patients should have their immunoglobulin profile determined at diagnosis and monitored every 2 years (patients <50 years) or every year (patients >50 years). If monoclonal gammopathy of undetermined significance (MGUS) is found, general MGUS guidelines should be followed. Future studies should focus on the utility of early treatment to prevent immunoglobulin abnormalities and multiple myeloma.