The multidisciplinary care of a woman presenting with lymphoma in pregnancy whose delivery was also complicated by placenta accreta spectrum.

We report the case of a 44-year-old presenting with breathlessness in her second trimester of pregnancy diagnosed with pulmonary diffuse large B cell lymphoma (DLBCL) which was further complicated by a placenta accreta spectrum (PAS) disorder. In pregnancy, she was treated with rituximab, cyclophosphamide, hydroxydaunorubicin, oncovin and prednisolone, which was associated with neutropenic sepsis requiring admissions to the intensive care unit with respiratory compromise. She safely delivered an infant at 31 weeks but required a hysterectomy at the time for PAS and seven days ventilation on the intensive care unit post-operatively. It is the first case report of DLBCL and PAS in pregnancy.

Solitary Iris Plasmacytoma With Anterior Chamber Crystalline Deposits.

PURPOSE: To report a case of solitary iris plasmacytoma successfully treated with ruthenium plaque radiotherapy. METHODS: A 44-year-old white woman presented with pain in the right eye and raised intraocular pressure. Her medical history included breast cancer treated 11 years earlier with lumpectomy, lymph node clearance, chemotherapy, and radiotherapy. On examination, the right iris had a tan-colored mass with crystalline deposits visible on the mass surface and on the corneal endothelium. The fundus and left eye were normal. An anterior segment ultrasound scan showed a mass with mixed internal echogenicity and internal blood flow. RESULTS: An iris biopsy showed an infiltrate of plasma cells. Immunohistochemistry for kappa and lambda light chains demonstrated lambda light chain restriction. Systemic hematologic investigations including complete blood count, serum paraproteins, bone marrow biopsy, and full-body magnetic resonance image were normal. The monoclonal plasma cell infiltrate was consistent with a solitary iris plasmacytoma. The mass was treated with ruthenium plaque radiotherapy. After 4 years of follow-up, the mass remained regressed, and no systemic myeloma has developed. CONCLUSIONS: Iris plasmacytoma is rare and should prompt systemic evaluation to rule out multiple myeloma. Solitary iris plasmacytoma can be successfully treated with plaque radiotherapy.

Dysfunction of the PI3 kinase/Rap1/integrin α(IIb)ß(3) pathway underlies ex vivo platelet hypoactivity in essential thrombocythemia.

Patients with myeloproliferative disorders (MPDs), such as essential thrombocythemia (ET) have increased risk of thrombosis and bleeding, which are major sources of morbidity and mortality. Most MPD patients have a gain of function mutation in Janus kinase 2 (JAK2V617F), but little is known how JAK2V617F affects platelet function. Here, we demonstrate that platelets from ET patients have impaired SFLLRN-mediated fibrinogen binding and have lost the potentiating effect of thrombopoietin (which couples to JAK2) on this pathway. In contrast, SFLLRN-mediated P-selectin expression, ATP secretion, phosphorylation of the PKC substrate pleckstrin, and Ca(2+) mobilization were unaffected in JAK2V617F positive platelets. In addition, thrombopoietin-mediated JAK2 phosphorylation was unchanged, suggesting that signaling pathways activated downstream of JAK2 are impaired. Indeed, we found that platelets from JAK2V617F positive ET patients have significantly reduced phosphorylation of the PI3 kinase substrate Akt, and have reduced activation of Rap1 in response to thrombopoietin, IGF-1,ADP, SFLLRN, and thrombin. This effect was independent of Giα P2Y12 purinergic receptor function as ADP-mediated inhibition of VASP phosphorylation was unchanged. These results demonstrate that the PI3 kinase/Rap1 pathway is intrinsically impaired in platelets from JAK2V617F-positive ET patients, resulting in diminished thrombin and thrombopoietin-mediated integrin α(IIb)ß(3) activation.

Stem cell donation--what advice can be given to the donor?

Haematopoietic stem cell transplantation (HSCT) is widely used to treat patients with a range of haematological and non-haematological disorders. Both bone marrow and peripheral blood stem cell collection are associated with morbidity and, very rarely, mortality. We investigated the information that exists to adequately inform donors about the relative merits of each procedure. We carried out a systematic review analysing data from six prospective randomised controlled trials of related donors and discuss here the merits and drawbacks of this approach. Registry data mostly describes patient outcome but stem cell donor registries collect and report information on unrelated donors which could easily be extended to related donors. Further well-designed, randomised studies are required.

Bone marrow harvest versus peripheral stem cell collection for haemopoietic stem cell donation in healthy donors.

BACKGROUND: Haemopoietic stem cells can be collected from a donor either as a bone marrow harvest or by peripheral blood collection. Both techniques have risks for the donor. OBJECTIVES: The aim of this review was to identify the adverse effects of haemopoietic stem cell donation and to compare the tolerability and safety of the two methods. SEARCH STRATEGY: We searched bibliographic databases including the Cochrane Central Register of Controlled trials (CENTRAL) (The Cochrane Library 2008, issue 2), MEDLINE and EMBASE up to May 2008. We also searched reference lists of articles and contacted experts in the field. SELECTION CRITERIA: Randomised controlled trials enrolling haemopoietic stem cell donors and evaluating the different methods of donating haemopoietic stem cells were eligible. DATA COLLECTION AND ANALYSIS: Two authors independently screened studies for inclusion. We extracted data and evaluated methodological quality. Quantitative analysis was not possible for most outcomes, but where used we preferred random-effects models due to the variability between the included studies. MAIN RESULTS: Six trials (807 donors) were eligible: all were substudies, or constituent parts of, larger randomised controlled trials of bone marrow and peripheral blood stem cell allogeneic transplantation. No included trial was designed solely to measure and assess the experience of stem cell donors. The donors in all studies were related to the stem cell recipient. Overall, both types of donors experienced pain subsequent to donation, and psychological morbidity. The trend was for bone marrow donors to experience more pain at the donation site, more overall adverse events, and more days of restricted activity. They were also more likely to require hospitalisation than peripheral blood stem cell donors. In contrast, peripheral blood stem cell donors experienced more pain prior to donation, which may be related to the pre-donation administration of granulocyte colony stimulating factor (G-CSF). The methodological quality of the studies was poor and indicated limitations due to the risk of selection and attrition bias. The proportion of donors from the parent trial not included in the donor substudies was also inadequately explained. AUTHORS' CONCLUSIONS: The different short-term morbidities associated with each type of haemopoietic stem cell donation were clear, with bone marrow donors experiencing more pain and more restriction post-donation than peripheral blood donors. However, the studies were limited by their methodological quality, failure to provide long-term follow up (for which larger numbers of donors would be required) and a failure to apply consistent measures of quality of life in a way which allows more meaningful evaluation across studies.