P279 THE ACUTELY ACL INJURED KNEE ASSESSED BY MRI-NATURAL HISTORY OF POST TRAUMATIC BONE MARROW LESIONS IN AN RCT OF SURGICAL VS. NON-SURGICAL TREATMENT

Mixed hematopoietic chimerism (MC) is a common finding after allogeneic bone marrow transplantation (BMT), but the natural history of this phenomenon remains unclear. To understand the evolution and the implications of this finding, we performed a prospective analysis of the development of mixed chimerism in 43 patients with hematologic malignancies who received bone marrow (BM) from human leukocyte antigen (HLA)-identical sibling donors. T-cell depletion in vitro with anti-T12 (CD6) monoclonal antibody and rabbit complement was used as the only method of graft-versus-host disease (GVHD) prophylaxis. Overall, MC was identified in peripheral blood (PB) and BM in 22 of 43 (51%) patients evaluated. MC was found by restriction fragment length polymorphism (RFLP) analysis in 21 of 40 (53%) patients, by cytogenetic analysis in 6 of 29 (21%) patients, and by red blood cell phenotyping in 4 of 9 (44%) patients. RFLP studies were performed at 0.5, 1, 3, 6, 9, and 12 months post-BMT and then every 6 months, and showed a high probability of developing MC in the first 6 months after BMT followed by stabilization after 12 months. Cytogenetic analysis was less sensitive in detecting MC. Once MC was detected after BMT, the percentage of recipient cells increased very slowly over more than 3 years of follow- up, and no patient reverted to complete donor hematopoiesis (CDH). Thus, recipient and donor cells remained in a relative state of equilibrium for prolonged periods that seemed to favor recipient cells over donor cells. Patient's disease, remission status, or intensity of the transplant preparative regimen did not influence the subsequent development of mixed chimerism. Early immunologic reconstitution was the only factor that correlated with the subsequent chimeric status of the patients. The percentage and absolute number of T3 (CD3) and T4 (CD4) positive cells at day 14 after BMT were significantly higher in the patients who maintained CDH but NK cell reconstitution was similar in both groups, suggesting that early reconstitution with T cells may play a role in preventing recovery of recipient cells after BMT. GVHD was also associated with maintenance of CDH, but the probability of relapse, survival, and disease-free survival was identical in patients with MC and CDH.

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The Natural History of Post-Traumatic Reflex Sympathetic Dystrophy

Journal of Hand Surgery (European Volume) ◽

10.1016/s0266-7681(98)80211-8 ◽

1998 ◽

Vol 23 (1) ◽

pp. 20-23 ◽

Cited By ~ 63

Author(s):

A. ZYLUK

Keyword(s):

Natural History ◽

Grip Strength ◽

Reflex Sympathetic Dystrophy ◽

Prospective Trial ◽

Signs And Symptoms ◽

Final Assessment ◽

Functionally Impaired ◽

History Of ◽

Post Traumatic

The paper presents the results of a prospective trial to examine the natural history of early reflex sympathetic dystrophy (RSD). Thirty patients with post-traumatic RSD of the hand were observed without treatment. They were reassessed 1, 2 and 6 months after diagnosis with a final assessment at 10 to 18 months (average 13 months). Twenty-seven patients completed the study. Three were withdrawn during the study because of persistence of signs and symptoms of RSD and were given further treatment. Of the 27 patients who completed the study, only one showed sufficient features of the condition to warrant the diagnosis of mild RSD. In the remaining 26, most features of RSD had resolved spontaneously. Pain and swelling disappeared more quickly than other features of RSD. Although the signs and symptoms of RSD had largely gone at 13 months, the hands were still functionally impaired because of weaker grip strength.

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Common knee conditions

10.1093/med/9780199550647.003.013020 ◽

2011 ◽

Author(s):

Shahryar Noordin ◽

Andrew Howard

Keyword(s):

Surgical Treatment ◽

Natural History ◽

Osteochondritis Dissecans ◽

Open Physes ◽

Lateral Dislocation ◽

Clinical Presentations ◽

Spine Pathology ◽

History Of ◽

Dislocation Of The Patella

♦ All children who complain of knee symptoms must be assessed for ipsilateral hip and spine pathology♦ Congenital or persistent lateral dislocation of the patella and obligatory dislocation of the patella have two different clinical presentations: surgical treatment (if required) is often complex♦ The natural history of stable osteochondritis dissecans lesions is generally favourable in a child with open physes.

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Natural History of 656 Patients with Non IgM MGUS.

Blood ◽

10.1182/blood.v104.11.4860.4860 ◽

2004 ◽

Vol 104 (11) ◽

pp. 4860-4860

Author(s):

Carolina Nobile ◽

Maria T. Petrucci ◽

Francesco Bartolozzi ◽

Anna Levi ◽

Marianna De Muro ◽

...

Keyword(s):

Bone Marrow ◽

Natural History ◽

Clinical History ◽

Cumulative Probability ◽

Rapid Progression ◽

Female Ratio ◽

Predictive Parameters ◽

Risk Of Progression ◽

History Of

Abstract A monoclonal gammopathy of undetermined significance (MGUS) occurs in about 1% of the population over 50 years of age. Of these, about 20% evolves in Multiple Myeloma (MM); however, so far, predictive parameters of progression have not yet been identified.The aim of this study was to analyse the natural history of a cohort of non IgM MGUS and to identify whether or not there were laboratory parameters at diagnosis which can be utilized as prognostic markers of stable MGUS or progression to MM. From February 1974 to July 2001, 656 non IgM MGUS, whose clinical history was concluded (lost to follow up or died), have been followed at the Hematology of the University “La Sapienza” in Rome. The duration of follow up ranged from 2 months to 324 months, male/female ratio was 1.14, median age was 65 years (range 19–92). In each patient we evaluated: hemoglobin, platelet count, serum protein electrophoresis, serum concentration of monoclonal protein, serum calcium, creatinine, uric acid, BUN and percentage of bone marrow plasma cells.A monoclonal component (MC) of IgG type was documented in 543 patients (83%) while in 106 (16%) it was of IgA type, 6 patients had biclonal MC and 1 had a λ light chain MC; BJ proteinuria was detected in 78 (11%) patients at diagnosis. After a median follow up of 60.1 months (range 2–324) the MC remained stable in 496 patients (75%), whereas in 160 cases (25%) increased to evolve in MM. According to the literature, cumulative probability of progression to MM was 3%, 7% and 17% at 5, 10 and 15 years respectively. Differently from what observed by other investigators, in this cohort of pts, the MGUS of IgA type was not associated with a higher risk of progression to MM. The median time of progression to MM was 60.7 months (range 3–256) and factors associated with a more rapid progression to MM were advanced age and a higher number of bone marrow plasmacells. At diagnosis of MM, the concentration of the serum MC was significantly higher (P<0.003) in patients who evolved from MGUS than in those with a newly diagnosed MM. None of the studied parameters at diagnosis of MGUS were predictive of evolution to MM even though, levels of MC <2.4 g/dl and bone marrow plasmacell infiltration <9% indicated a slower progression to overt MM.

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A Global Registry of Patients with Paroxysmal Nocturnal Hemoglobinuria.

Blood ◽

10.1182/blood.v114.22.3007.3007 ◽

2009 ◽

Vol 114 (22) ◽

pp. 3007-3007

Author(s):

Robert Brodsky ◽

Hubert Schrezenmeier ◽

Petra Muus ◽

Monica Bessler ◽

Jeffrey Szer ◽

...

Keyword(s):

Bone Marrow ◽

Natural History ◽

Board Of Directors ◽

Research Funding ◽

Health Care Services ◽

Clinical Symptoms ◽

Marrow Transplant ◽

Advisory Committees ◽

Care Services ◽

History Of

Abstract Abstract 3007 Poster Board II-983 Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal hematopoietic stem cell disease characterized by complement-mediated hemolysis which can lead to life-threatening complications including thrombosis, kidney disease, and pulmonary hypertension. The natural history of PNH is highly variable and has previously been captured by retrospective assessment. However, the clinical presentation and prognosis of the disease has changed with the increased awareness of PNH, the increased use of more sensitive diagnostic tests, and the availability of new treatment. Specifically, the development of targeted but potentially life-long therapies, such as terminal complement blockade, necessitates the collection of long-term outcomes data in this patient population. We have established a global PNH Registry in order to redefine the natural history of PNH capturing a wide range of patients from all over the world. The goal of the present analysis is to describe the data collected for the patients in the Registry and demonstrate its use as an ongoing repository of information on symptoms, course, complications and treatment in patients with a PNH clone. The first patient was enrolled in January 2005, with data contributed from 62 clinical sites in 12 countries on 4 continents as of July 2009. Patients are included in the Registry regardless of amount of clone, bone marrow pathology, symptoms, or treatments. Sites collect data at enrollment and every 6 months including demographics, diagnostics and flow cytometry, other lab tests including LDH, medical conditions such as bone marrow pathology and major adverse vascular events (MAVE), clinical symptoms, medications and transfusions, qualitative assessments, bone marrow transplant, and mortality. Patients complete a questionnaire every 6 months including health-related quality-of-life, symptoms, and use of health care services. As of July 2009 there were 368 enrolled patients in the Registry (51% female, 49% male). Mean age at enrollment was 43.6 ±16.7, while mean age at first PNH symptoms was 35.9±16.7. At enrollment, median GPI-deficient granulocyte percentage (GPI-DG) was 80.4%, while 10% of patients had a GPI-DG <10. Of those patients with a GPI-DG <10, 81% had bone marrow pathology (62% with aplastic anemia, 16% with myelodysplastic syndrome, 3% other pathology) compared to 38% of patients with GPI-DG 350. MAVE was increased in patients with GPI-DG 350 compared to <10 (22% vs. 8%), as were LDH levels (median 1042 vs. 239 U/L). Patients with GPI-DG <10 reported high levels of significant clinical symptoms (fatigue 59%; dyspnea 52%; abdominal pain 41%) and symptom reporting was generally increased in patients with higher GPI-DG levels. Treatment in the year prior to Registry enrollment primarily consisted of transfusions (42%), anticoagulation therapy (30%), eculizumab (29%), and immunosuppression (23%), although these varied by GPI-DG level. Clinicians assessed 14% of patients with a Karnofsky score of 70 or lower (i.e., not capable of work or normal activity). Patients' assessment of their overall health, social functioning, and fatigue worsened and use of health care services increased with higher GPI-DG. At this time, median follow up is 12.8 months, although 25% of patients have been followed for at least 30 months. Two patients received a bone marrow transplant and 8 are deceased. In conclusion, preliminary data show that greater GPI-DG is associated with less underlying bone marrow pathology, more hemolysis, more thromboses, and more patient-reported symptoms. New clinical sites and geographic regions are encouraged to participate in the Registry ([email protected]). This global PNH Registry should help to redefine prospectively the long-term natural history of PNH, its treatments, and the outcomes of treatment. Disclosures: Brodsky: Alexion: Membership on an entity's Board of Directors or advisory committees. Schrezenmeier:Alexion: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding. Muus:Alexion: Membership on an entity's Board of Directors or advisory committees. Bessler:Alexion: Membership on an entity's Board of Directors or advisory committees. Szer:Alexion: Membership on an entity's Board of Directors or advisory committees. Rotoli:Author Deceased: Author Deceased. Maciejewski:Celgene: Speakers Bureau; Gemzyne: Research Funding; Taligen: Membership on an entity's Board of Directors or advisory committees. Socie:Alexion: Consultancy, Membership on an entity's Board of Directors or advisory committees. Urbano-Ispizua:Alexion: Membership on an entity's Board of Directors or advisory committees. Rosse:Alexion: Membership on an entity's Board of Directors or advisory committees. Karnell:Alexion: Employment. Bedrosian:Alexion Pharmaceuticals: Employment, Equity Ownership. Hillmen:Alexion Pharmaceuticals: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding.

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