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1.  Ophthalmic manifestations of herpes zoster virus in patients with multiple myeloma following bone marrow transplantation 
BMJ Case Reports  2013;2013:bcr2012007625.
We report three patients with ophthalmic herpes zoster (HZ) manifestations on the background diagnosis of multiple myeloma (MM). It seems that immunocompromised status has caused reactivation of the varicella zoster virus (VZV) producing a well-characterised neurological syndrome and subsequent postherpetic neuralgia in two patients. One patient experienced lymphocytic leptomeningitis resulting in unilateral optic neuritis. All patients received similar myeloma disease-specific treatment prior to HZ reactivation. All patients were treated with thalidomide and steroids, and they thereafter underwent autologous stem cell transplantation. Prior to HZ reactivation they received new immunomodulatory drugs in the form of thalidomide in addition to bortezomib (2 patients) and lenalidomide (1 patient). Immediate specific antiviral therapy was successfully applied with intravenous acyclovir for 10 days, followed by long-term oral famciclovir maintenance. Two patients progressed to have chronic HZ ophthalmicus and postherpetic neuralgia requiring ongoing antiviral therapy and neuroepileptic medications for the neuropathic pain.
doi:10.1136/bcr-2012-007625
PMCID: PMC3603998  PMID: 23429015
2.  Review of Management and Outcomes in Women with Thrombophilia Risk during Pregnancy at a Single Institution 
ISRN Obstetrics and Gynecology  2014;2014:381826.
Pregnancy is a hypercoagulable state associated with an increased risk of venous thromboembolic disease (VTE). We retrospectively studied 38 Caucasian pregnant women with thrombophilia risk and compared their obstetric outcomes with a matched cohort without known thrombophilia risk during the period between January 2007 and December 2010. There were (2) cases with factor V Leiden, (6) prothrombin gene mutation, (1) antithrombin III deficiency, (2) protein C deficiency, (3) protein S deficiency, (10) MTHFR mutation, (7) anti-cardiolipin antibodies, and (1) lupus anticoagulant. Patients without thrombophilia who presented with recurrent unprovoked VTE were considered as high risk (6 cases). Most patients received anticoagulation (34/38) with aspirin only (6), enoxaparin (27), and warfarin (1). Twenty-six out of thirty-eight pregnant women (68.4%) with an increased risk of thrombophilia experienced one or more obstetric complications defined as hypertension, preeclampsia, placenta abruptio, VTE, and oligohydramnios, compared with 15 out of 40 (37.5%) pregnant women in the control group (OR 3.6; 95% CI 1.42, 9.21, P < 0.001). The incidence of obstetric complications was significantly higher in the thrombophilia group compared to the controls. However, these complications were the lowest among patients who received full-dose anticoagulation. Our study suggests that strict application of anticoagulation therapy for thrombophilia of pregnancy is associated with an improved pregnancy outcome. The study was registered in the Australian and New Zealand Clinical Trials Registry under ACTRN12612001094864.
doi:10.1155/2014/381826
PMCID: PMC3945432  PMID: 24693443
3.  Assessment of whole body MRI and sestamibi technetium-99m bone marrow scan in prediction of multiple myeloma disease progression and outcome: a prospective comparative study 
BMJ Open  2013;3(1):e002025.
Objectives
This study aims primarily to determine whether whole body MRI (WB-MRI) and Sestamibi Technetium-99m-bone marrow (MIBI) scans in the same patients produce the same estimate of disease load and location, and secondly, to study possible association between the bone disease detected by these scans and the effect on disease outcome and survival. Bone disease occurs in about 90% of multiple myeloma (MM) patients. There are no data comparing the new diagnostic modalities with WB-MRI and MIBI in MM.
Design
A prospective comparative study between WB-MRI and MIBI scans in assessing bone disease and outcome of MM.
Participants and methods
Sixty-two consecutive patients with confirmed MM underwent simultaneous WB-MRI (both axial T1 and turbo spin echo short tau inversion recovery (STIR)) and MIBI scans at a single institution from January 2010 to January 2011, and their survival status was determined in January 2012. The median age was 62 years (range 37–88) with a male-to-female ratio of 33 : 29.
Results
In vertebrae and long bones, MRI scan detected more disease compared with MIBI scan (p<0.001) but there was less difference in the skull (p=0.09). In the ribcage, the MIBI scan detected more lytic lesions of the ribs compared with MRI scan (p<0.001). Thirteen of the 62 patients died during the 24-month follow-up. Increased disease detected in all bones by both scans was associated with increased mortality risk (MIBI p=0.001; MRI-STIR p=0.044; but not MRI-T1 p=0.44). In all combined bone groups, the mean MIBI scan results provided a better prediction of mortality than MRI scan over the follow-up period (MRI-T1 vs MIBI p=0.019; MRI-STIR vs MIBI p=0.047).
Conclusions
Although WB-MRI detected more MM bone disease, MIBI scan predicted overall disease outcome and mortality better than MRI scan. Further studies to define optimum use of these imaging techniques are warranted.
Trial registration number
The study was registered prospectively in the Australian and New Zealand Clinical Trials Registry at http://www.ANZCTR.org.au under No: ACTRN12609000761268.
doi:10.1136/bmjopen-2012-002025
PMCID: PMC3549203  PMID: 23315438
Nuclear Medicine; Radiology & Imaging; Qualitative Research
4.  Early Application of High Cut-Off Haemodialysis for de-Novo Myeloma Nephropathy is Associated with Long-Term Dialysis-Independency and Renal Recovery 
Background
Multiple myeloma (MM) is a haematological malignancy associated with kidney injury resulting from cast nephropathy, which can be caused by monoclonal free light chains (FLC). It has been demonstrated that early reduction of FLC can lead to a higher proportion of patients recovering renal function with a better outcome, especially if high cut-off haemodialysis (HCO-HD) combined with chemotherapy is used.
Patients and Methods
In this study, four cases with MM nephropathy were treated with HCO-HD and chemotherapy at a single institution during the period from August 2009 to August 2011. All of the patients presented with acute renal failure and high serum FLC. All patients underwent a bone marrow biopsy to confirm the diagnosis of MM, according to the WHO criteria. Three patients had de novo MM and one patient had relapsed light chain myeloma disease. All patients underwent HCO-HD concomitantly with specific myeloma therapy once the diagnosis or relapse of MM was established.
Results
After a medial follow up of 26 months, (range, 13–36) our data showed that all patients had a significant decrease in serum FLC through HCO-HD, proving the effectiveness of HCO-HD in managing MM. De-novo MM patients restored their renal function and achieved low-level FLC early in the treatment and became dialysis-independent. One patient with relapsed myeloma remained dialysis-dependent.
Conclusion
In summary, our study suggests that in myeloma nephropathy associated with light-chain MM, HCO-HD should be initiated as early as possible. At the same time a specific MM treatment should be initiated to gain control of the disease and salvage the kidneys in order to achieve dialysis-independency. Further randomized trials to confirm our results are warranted.
doi:10.4084/MJHID.2013.007
PMCID: PMC3552781  PMID: 23350020
5.  Three-year follow-up of a randomised clinical trial of intravenous versus oral iron for anaemia in pregnancy 
BMJ Open  2012;2(5):e000998.
Background
To date, there are no data available concerning the impact of iron therapy on the long-term well-being and health-related quality of life (HRQoL) in pregnancy.
Objective
To assess the long-term effect of iron therapy on HRQoL in pregnancy.
Design
This is a follow-up study conducted between January 2010 and January 2011 of an earlier randomised open-label clinical trial of intravenous and oral iron versus oral iron for pregnancy-related iron deficiency anaemia. We used a modified version of the SF-36 questionnaire together with the original prospective HRQoL data collected during and after pregnancy.
Participants and interventions
Of the original evaluable 183 pregnant Caucasian women randomised to receive oral iron or a single intravenous iron polymaltose infusion followed by oral iron maintenance, 126 women completed the follow-up HRQoL study.
Methods
The participants were followed up 4 weeks after treatment, predelivery and postdelivery for a median period of 32 months (range, 26–42) with a well-being and HRQoL questionnaire using a modified SF-36 QoL-survey and child growth charts as set by the Australasian Paediatric Endocrine Group (APEG).
Results
Patients who received intravenous iron demonstrated significantly higher haemoglobin and serum ferritin levels (p<0.001). There were strong associations between iron status and a number of the HRQoL parameters, with improved general health (p<0.001), improved vitality (physical energy) (p<0.001), less psychological downheartedness (p=0.005), less clinical depression (p=0.003) and overall improved mental health (p<0.001). The duration of breastfeeding was longer (p=0.046) in the intravenous iron group. The babies born in both groups recorded similarly on APEG growth chart assessments.
Conclusions
Our data suggest that HRQoL is improved until after pregnancy in anaemic pregnant women by repletion of their iron stores during pregnancy. About 80% of the intravenous iron group showed a maintained normal ferritin until delivery with long-term benefits. Further studies to confirm these findings are warranted.
doi:10.1136/bmjopen-2012-000998
PMCID: PMC3488743  PMID: 23087011
Qualitative Research
6.  Iron Deficiency Anaemia in Pregnancy and Postpartum: Pathophysiology and Effect of Oral versus Intravenous Iron Therapy 
Journal of Pregnancy  2012;2012:630519.
Nutritional iron-deficiency anaemia (IDA) is the most common disorder in the world, affecting more than two billion people. The World Health Organization's global database on anaemia has estimated a prevalence of 14% based on a regression-based analysis. Recent data show that the prevalence of IDA in pregnant women in industrialized countries is 17.4% while the incidence of IDA in developing countries increases significantly up to 56%. Although oral iron supplementation is widely used for the treatment of IDA, not all patients respond adequately to oral iron therapy. This is due to several factors including the side effects of oral iron which lead to poor compliance and lack of efficacy. The side effects, predominantly gastrointestinal discomfort, occur in a large cohort of patients taking oral iron preparations. Previously, the use of intravenous iron had been associated with undesirable and sometimes serious side effects and therefore was underutilised. However, in recent years, new type II and III iron complexes have been developed, which offer better compliance and toleration as well as high efficacy with a good safety profile. In summary, intravenous iron can be used safely for a rapid repletion of iron stores and correction of anaemia during and after pregnancy.
doi:10.1155/2012/630519
PMCID: PMC3389687  PMID: 22792466
7.  Successful Treatment with Thrombopoietin Receptor Agonist in Avoiding Splenectomy for Patients with Chronic Refractory Immune Thrombocytopenia 
Background
Chronic immune thrombocytopenia (ITP) is a condition associated with significant morbidity; however the management options are often unsatisfactory with a portion of patients exhibiting a refractory-relapsing disease path despite various lines of treatment including splenectomy. As a thrombopoietin receptor agonist, eltrombopag (GlaxoSmithKline, Australia) provides a novel treatment option for patients with refractory disease. We describe the outcomes of four patients with chronic ITP, who were treated with eltrombopag as a single agent.
Methods
Four Caucasian patients with chronic refractory ITP (2 males; 2 females) were enrolled in this study with a mean age of 48 years (range, 39–59). All patients were non-splenectomised and were refractory to several lines of treatment including steroids, intravenous immunoglobulin, vincristine, and azathioprine, one patient has also received rituximab (a monoclonal antibody that binds the CD20 antigen expressed by B-lymphocytes). All patients were treated with oral eltrombopag (50–75 mg) for a median period of 12 months (range, 9–16).
Results
After a median follow up of 20 months (range, 11–34), platelet counts recovered to normal levels in two patients. One recovered a normal platelet count after 13 months, the other 34 months of completion of treatment with eltrombopag. No additional immune suppressive therapy was required.
The other two patients also discontinued eltrombopag at 27 and 11 months after achievement of satisfactory platelet counts above 30/nL without any bleeding complications. Other forms of immune therapy were also ceased in these two cases. None of the four patients required splenectomy.
Conclusion
The clinical outcomes in this small cohort of patients suggests that eltrombopag may have a role to play in the long term control of chronic ITP whilst avoiding splenectomy and long term immunosuppressive therapy. The beneficial outcomes in our patients led to a sustained elevation in platelets with no adverse effects noted when used for relatively longer periods than previously reported. It is worth noting that spontaneous remission does occur with ITP and is the most likely cause for the favourable outcome with eltrombopag therapy. However, if eltrombopag is able to reduce the need for splenectomy in patients with chronic ITP then a distinct quality of care outcome can be achieved by avoiding the recognised short- and long-term complications of splenectomy. Randomised controlled trials with long-term follow up are warranted.
doi:10.4084/MJHID.2012.003
PMCID: PMC3279318  PMID: 22348185
8.  An Unusual Presentation of Desmoplastic Small Round Cell Tumour of the Abdomen: Morphological, Immunohistochemical, Ultrastructural, and Molecular Studies 
Desmoplastic small round cell tumour (DSRCT) is an aggressive and a rare neoplasm. We report on a 34-year-old male who had abdominal discomfort with a large intraperitoneal mass. Histological examination of the tumour biopsy revealed sheets of small round cells. The cells were positive with vimentin and desmin (with occasional dot positivity) and negative for WT1 and CD 99 with immunohistochemistry. Cytogenetics showed a translocation disrupting the EWSR 1 gene on 22 q 12 consistent with DSRCT. Electron microscopic examination showed sparse cytoplasmic organelles. The patient succumbed 34 months from disease presentation after multiple chemotherapies and thereafter radiotherapy. In summary, our case exemplifies that it is crucial to combine clinical, histological, and molecular aspects in diagnosing DSRCT especially when characteristic dot positivity with desmin is weak along with deficient marking of WT1 and CD99 by immunohistochemistry. Histology was also less clear than published examples of this entity with a poor desmoplastic response. A multidisciplinary approach including early referral to specialised centres is recommended in these cases as tertiary referral centres will be required to substantiate the diagnosis.
doi:10.1155/2011/183938
PMCID: PMC3350138  PMID: 22606441
9.  Primary Breast Lymphoma: A Rare Entity 
In the past two decades there has been an increase in the incidence of Non-Hodgkin Lymphoma and Hodgkin disease. This has been accompanied by an increase in the numbers of extranodal lymphoma. Despite this primary breast lymphoma is a rare disease. We present a case of a 74 year old female with primary breast lymphoma. Methods of imaging including PET/CT are discussed. Criteria for diagnosing primary breast lymphoma are presented. In addition diagnostic methods and therapeutic options are considered.
doi:10.3941/jrcr.v5i5.750
PMCID: PMC3303444  PMID: 22470790
Breast Imaging; Lymphoma; Breast Lymphoma; Breast Cancer
10.  High Serum Erythropoietin and Ferritin Levels in Conjunction with Anemia Response in Malignant Lymphoma 
Anemia is a common finding in lymphoma. There are few data available regarding the erythropoietin (EPO) levels in conjunction with ferritin in lymphoma patients. We prospectively evaluated 55 patients diagnosed with malignant lymphoma during the period between November 2006 and March 2008 at the King Abdullah University Teaching Hospital, Jordan. Our data showed that 74.4% of lymphoma patients were anemic. Furthermore, serum EPO and ferritin levels were higher in lymphoma patients compared with the healthy controls (P=0.001). The observed versus predicted EPO ratio showed also significantly higher levels in anemic lymphoma patients compared to healthy controls (p=0.03). There was an improvement in the Hb level in lymphoma patients who were treated with at least 3-cycles of chemotherapy as compared with newly-diagnosed patients. An adequate increase of EPO levels was observed in anemic lymphoma patients and notably associated with higher ferritin levels and improvement of Hb (p<0.001).
Our findings suggest that ferritin estimation in lymphoma patients may predict the level of erythropoiesis and possibly the degree of anemia. Further studies to confirm these findings are warranted.
doi:10.4084/MJHID.2011.018
PMCID: PMC3113282  PMID: 21713081
11.  Extramedullary Plasmacytoma of the Tonsil with Nodal Involvement 
We present a rare case of extramedullary plasmacytoma of the palatine tonsil with cervical lymph node involvement treated by surgical resection. A 58-year-old Caucasian male presented with a solitary 3 cm × 3 cm jugulodigastric lymph node and was found to have an ipsilateral tonsillar swelling. The involved tonsil and lymph node were surgically resected after two inconclusive fine-needle aspirates, and plasmacytoma was confirmed histologically and by immunocytochemistry. Adjuvant radiotherapy was not indicated as adequate resection was achieved at surgery. We also highlight the challenges of diagnosis when fine-needle aspiration is inconclusive and the need for careful planning before surgery.
doi:10.1155/2010/302656
PMCID: PMC2913787  PMID: 20706681
12.  Effect of Immunoglobulin Therapy on the Rate of Infections in Multiple Myeloma Patients Undergoing Autologous Stem Cell Transplantation or Treated with Immunomodulatory Agents. 
Multiple myeloma (MM) is associated with a significant risk of infection due to immune dysfunction. Infections are a major cause of morbidity and mortality in MM patients. There are few data available regarding the prevalence of infection in MM patients, especially in conjunction with newer generations of immunomodulatory drugs (thalidomide, bortezomib, lenalidomide) or post autologous stem cell transplantation (ASCT). Intravenous immunoglobulin (IVIG) has been used successfully to reduce infection rates in the stable phase of MM, with limited data in other stages.
We retrospectively analyzed 47 patients with MM from March 2006 to June 2009 at our institution. All patients received thalidomide and steroid therapy for at least 6 months. Nine patients received bortezomib and 11 lenalidomide subsequent to thalidomide, because of disease progression, and 22 patients underwent ASCT. The median age was 64 years (range 37–86), with a female–to-male ratio of 18:29. The median residual-serum IgG-level at time of infection was 3.2 g/L, IgA 0.3 g/L and IgM 0.2 g/L. Most patients suffered from recurrent moderate to severe bacterial infections, including the ASCT group. Fifteen patients suffered from different degrees of viral infections.
All patients except 3 received IVIG therapy with a significant decline of the rate of infection thereafter (p<001). Our analysis shows that patients with MM treated with the new immunomodulatory drugs in conjunction with steroids are at significant increased risk of infection. Employing IVIG therapy appears to be an effective strategy to prevent infection in this cohort of patients. Further studies to confirm these findings are warranted.
doi:10.4084/MJHID.2010.005
PMCID: PMC3033112  PMID: 21415947

Results 1-13 (13)