Life-Threatening Acute Chest Syndrome in a Patient With Sickle Cell Disease After Switching From Hydroxyurea Therapy to Partial Exchange Transfusions: A Case Report

Acute chest syndrome (ACS) is a severe form of vaso-occlusive crisis, which is a main feature of sickle cell disease (SCD), an inherited hemoglobinopathy. Traditionally, hydroxyurea has been the treatment of choice for SCD to prevent vaso-occlusive crises including ACS. However, hydroxyurea may be contraindicated, for example, in patients wanting to have children. We here present a young male with SCD who wanted to become a father and developed a life-threatening episode of ACS following discontinuation of hydroxyurea and switching to partial exchange blood transfusions. The patient, aged 32 years and originally from Bahrain, had been diagnosed with homozygous SCD, alpha-thalassemia, and glucose-6-phosphate dehydrogenase deficiency as a child. He had an episode of ACS with moderate severity in 2008, after which he started using hydroxyurea. From 2008 until the present, he did not experience any episodes of ACS. About six months before the present episode, he stopped using hydroxyurea and switched to partial exchange transfusions, aiming to keep hemoglobin S (HbS) below 30%. The interval between the transfusions was typically about seven to eight weeks. On the evening (day 1) before hospital admission, he developed typical symptoms and signs of vaso-occlusive crisis, and during the first day in the hospital (HbS about 55%), his pulmonary function deteriorated, and he also developed cerebral symptoms (somnolence and confusion). On suspicion of ACS, a full blood exchange transfusion was administered on day 3. He then gradually recovered clinically, and his laboratory values also normalized. He was discharged on day 10. Subsequent follow-up visits at the outpatient clinic the following month were unremarkable. Possibly, this severe episode of ACS was triggered by switching from hydroxyurea therapy to partial exchange transfusions with too long intervals between the transfusions. This novel case is a compelling reminder of the possible perils that may accompany the discontinuation of hydroxyurea, the best-documented therapy in SCD

Serum calcium is an independent predictor of quality of life in multiple myeloma

Bone disease is an important feature of multiple myeloma, and hypercalcaemia is a frequent complication of this disease. We examined the association between serum calcium and quality of life (QOL) scores of 686 multiple myeloma patients at the time of diagnosis. Data from two Nordic studies using the EORTC QLQ-C30 questionnaire were analysed by means of linear regression analysis and a curve fitting program. Serum calcium was independently related to appetite loss, nausea/vomiting and physical functioning (P < 0.001) and to cognitive functioning (P = 0.001), i.e. scores reflecting symptoms that are well known in non-malignant hypercalcaemia. In addition, we found a highly significant independent relationship between serum calcium and the scores for fatigue and pain (P < 0.001). Serum calcium appeared to be as strong a predictor for fatigue as the concentration of haemoglobin. A cubic model (y = a + bx(3)) fitted the data slightly better than the simple linear model (y = a + bx) and suggested worsening QOL scores at levels of serum calcium above 2.5-3.0 mmol/L. Hypercalcaemia in patients with multiple myeloma seems to be associated with the same symptoms as in non-malignant hypercalcaemia. In addition, an increased level of serum calcium may aggravate the pain and fatigue caused by the skeletal disease itself

Monoklonal gammopati av klinisk betydning

Monoklonal gammopati er hyppig og kan være assosiert med alvorlig sykdom, men målrettet behandling kan gi betydelig helsegevinst. Det er godt kjent at funn av monoklonal gammopati bør føre til en vurdering om pasienten kan ha myelomatose eller annen B-lymfoproliferativ sykdom, men at det monoklonale immunglobulinet direkte kan føre til sykdom, er langt mindre kjent

Complement activation is a crucial pathogenic factor in catastrophic antiphospholipid syndrome.

Research suggests that the complement system plays a role in the pathophysiology of APS, and experimental studies have revealed a requirement for C5 in APS-induced thrombus formation [1]. However, limited data are available on complement activation in the potentially life-threatening catastrophic form of APS (CAPS) [2]. Despite occasional reports of beneficial clinical effects of the complement inhibitor eculizumab in CAPS [3], the degree and pattern of complement activation has not been investigated. Such information is required as a rationale for therapeutic complement inhibition and is provided in the present report

Bortezomib consolidation after autologous stem cell transplantation in multiple myeloma: a Nordic Myeloma Study Group randomized phase 3 trial

The Nordic Myeloma Study Group conducted an open randomized trial to compare bortezomib as consolidation therapy given after high-dose therapy and autologous stem cell transplantation (ASCT) with no consolidation in bortezomib-naive patients with newly diagnosed multiple myeloma. Overall, 370 patients were centrally randomly assigned 3 months after ASCT to receive 20 doses of bortezomib given during 21 weeks or no consolidation. The hypothesis was that consolidation therapy would prolong progression-free survival (PFS). The PFS after randomization was 27 months for the bortezomib group compared with 20 months for the control group (P = .05). Fifty-one of 90 patients in the treatment group compared with 32 of 90 controls improved their response after randomization (P = .007). No difference in overall survival was seen. Fatigue was reported more commonly by the bortezomib-treated patients in self-reported quality-of-life (QOL) questionnaires, whereas no other major differences in QOL were recorded between the groups. Consolidation therapy seemed to be beneficial for patients not achieving at least a very good partial response (VGPR) but not for patients in the >= VGPR category at randomization. Consolidation with bortezomib after ASCT in bortezomib-naive patients improves PFS without interfering with QOL. This trial was registered at www.clinicaltrials.gov as #NCT00417911