Monoclonal Gammopathy of the Immunoglobulin a Class in a Two-Year-Old Girl with Ataxia Telangiectasia

1998 ◽  
Vol 1 (4) ◽  
pp. 319-321 ◽  
Author(s):  
P.S. McDonald ◽  
D. Cora-Bramble ◽  
L. De Palma
Keyword(s):  
Ataxia Telangiectasia ◽  
Monoclonal Gammopathy ◽  
Unusual Case ◽  
Immunoglobulin A ◽  
Normal Serum ◽  
Neurologic Disorder ◽  
A Value ◽  
First Case ◽  
Immunofixation Electrophoresis ◽  
Restricted Mobility

Ataxia telangiectasia is an autosomal recessive neurologic disorder which is frequently associated with a deficiency of IgA immunoglobulin. We report an unusual case of monoclonal gammopathy of the IgA kappa type in a 2-year-old female patient newly diagnosed with ataxia telangiectasia. Quantitative analysis of the patient's immunoglobulins revealed a marked elevation in the IgA fraction with a value of 672 mg/dL (normal 14–123 mg/dL). The IgG and IgM fractions were normal. Serum protein electrophoresis showed a band of restricted mobility present in the gamma region, which was identified as a monoclonal IgA kappa immunoglobulin on immunofixation electrophoresis. This is the first case report of a patient with ataxia telangiectasia associated with an IgA monoclonal gammopathy.

A Giant Coronary Sinus Aneurysm with Secondary Vena Cava Aneurysms

2016 ◽  
Vol 19 (1) ◽  
pp. 028
Author(s):  
Shengjun Wu ◽  
Peng Teng ◽  
Yiming Ni ◽  
Renyuan Li
Keyword(s):  
Computed Tomography ◽  
Heart Transplantation ◽  
Coronary Sinus ◽  
Unusual Case ◽  
Superior Vena ◽  
Vena Cava ◽  
Final Diagnosis ◽  
Rare Entity ◽  
First Case ◽  
Enhanced Computed Tomography

Coronary sinus aneurysm (CSA) is an extremely rare entity. Herein, we present an unusual case of an 18-year-old symptomatic female patient with a giant CSA. Secondary vena cava aneurysms were also manifested. The final diagnosis was confirmed by enhanced computed tomography (CT) and cardiac catheterization. As far as we know, it is the first case that such a giant CSA coexists with secondary vena cava aneurysms. Considering the complexity of postoperative reconstruction, we believe that heart transplantation may be the optimal way for treatment. The patient received anticoagulant due to the superior vena cava (SVC) thrombosis while waiting for a donor.

scholarly journals Intraductal papillary mucinous neoplasm of the oesophagus: an unusual case of dysphagia

2012 ◽  
Vol 94 (2) ◽  
pp. e92-e94 ◽  
Author(s):  
E Crighton ◽  
A Botha
Keyword(s):  
Surgical Resection ◽  
Intraductal Papillary Mucinous Neoplasm ◽  
Unusual Case ◽  
Heterotopic Pancreas ◽  
Common Symptom ◽  
Ivor Lewis ◽  
Mucinous Neoplasm ◽  
Extensive Search ◽  
First Case ◽  
Oesophageal Wall

We report the case of a 58-year-old woman presenting with dysphagia secondary to an intraductal papillary mucinous neoplasm arising from a heterotopic pancreas in the oesophageal wall. This was successfully treated with a laparoscopic/thoracoscopic ivor Lewis oesophagectomy. Dysphagia is the most common symptom of oesophageal tumours regardless of aetiology of the tumour and can be treated successfully with surgical resection. Through an extensive search of the literature, we found that a heterotopic pancreas in the oesophagus is extremely rare with only ten cases being reported. We describe what we believe to be the first case of a heterotopic pancreas in the oesophagus transforming into an intraductal papillary mucinous neoplasm.

scholarly journals Multiple Myeloma with Suspected Non-Secretory Type

2020 ◽  
Vol 27 (1) ◽  
Author(s):  
Annisa Ginar Indrarsi ◽  
Usi Sukorini
Keyword(s):  
Multiple Myeloma ◽  
Bone Marrow ◽  
Monoclonal Gammopathy ◽  
Plasma Cells ◽  
Immunoglobulin A ◽  
Protein Electrophoresis ◽  
Palpebral Conjunctiva ◽  
General Weakness ◽  
M Spike ◽  
G Ratio

Multiple Myeloma (MM) is a hematological malignancy characterized by clonal plasma cell in bone marrow that produceabnormal globulin, which resulted in monoclonal gammopathy. Multiple Myeloma Non-Secretory (MMNS) is a very rareform of multiple myeloma with monoclonal plasmocytic proliferation in bone marrow supported by clinical manifestationand radiological findings. However, plasma cells fail to secrete immunoglobulin. A 44-year-old female came to SardjitoGeneral Hospital with main complaints of weakness and back pain. General weakness and pale palpebral conjunctiva were6 observed (+/+), liver and spleen were not palpable. Blood test results were as follows: Hb 3.0 g/dL, RBC 1.07 x 10 / μL, WBC3 3 562 x 10 /μL, PLT 114 x 10 /μL, A/G ratio 1.07, BUN 51.5 mg/dL, creatinine 4.62 mg/dL, and calcium 3.1 mmol/L. Skeletalsurvey suggested a multiple osteolytic. Protein electrophoresis revealed hypogammaglobulinemia with no M-spike. Therewere 66% of plasma cells in bone marrow. Patient was diagnosed by MMNS. Diagnosis MMNS can be established if clonalplasmacytes is accompanied with renal insufficiency and hypercalcemia. However, monoclonal gammopathy was not foundin serum protein electrophoresis. A case reported of 44-year-old female diagnosed as MMNS with 'punched out' multipleosteolytic, increased plasma cells in bone marrow without evidence of paraprotein in circulation proved by low A/G ratio andnegative M-spike.

Nucleosynthesis

1986 ◽  
Vol 320 (1556) ◽  
pp. 557-564 ◽  
Keyword(s):  
Light Element ◽  
Hubble Constant ◽  
Big Bang ◽  
Element Abundances ◽  
Stellar Nucleosynthesis ◽  
Baryonic Density ◽  
Redshift Surveys ◽  
A Value ◽  
First Case ◽  
The Universe

Both Big-Bang and stellar nucleosynthesis have outcomes related to the density of baryonic matter, but whereas in the first case there is a standard model that makes very precise predictions of light element abundances as a function of the mean density of baryons in the Universe, in the second case various uncertainties permit only very limited conclusions to be drawn. As far as Big-Bang synthesis and the light elements are concerned, existing results on D, 3 He and 7 Li indicate a value of Ω N h 2 0 greater than 0.01 and less than 0.025, where Ω N is the ratio of baryonic density to the closure density and h 0 is the Hubble constant in units of 100 km s -1 Mpc -1 ; probably 0.5 < h 0 < 1. New results on the primordial helium abundance give a still tighter upper limit to Ω N ,Ω N h 2 0 < 0.013, which when compared with redshift surveys giving Ω > 0.05 implies that the observed matter can all be baryonic only if the various uncertainties are stretched to their limits.

Intramedullary tuberculoma of the spinal cord

1999 ◽  
Vol 90 (1) ◽  
pp. 125-128 ◽  
Author(s):  
John K. Ratliff ◽  
Edward S. Connolly
Keyword(s):  
Spinal Cord ◽  
World Literature ◽  
Unusual Case ◽  
Spinal Tuberculosis ◽  
Tuberculosis Infection ◽  
Hiv Positive ◽  
Content Type ◽  
First Case ◽  
Immunodeficiency Virus ◽  
Hiv Negative

✓ Intramedullary spinal tuberculosis infection remains an extremely rare disease entity. In the most recent reviews only 148 cases have been reported in the world literature, although numerous recent reports from developing countries and on human immunodeficiency virus (HIV)—positive patients have increased this number. The authors present an unusual case of intramedullary tuberculoma in an HIV—negative patient from the southern United States who demonstrated no other signs or symptoms of tuberculosis infection. The authors believe that this is the first case of its kind to be presented in recent literature. The presentation of miliary disease via an isolated intramedullary spinal mass in a patient with no evident risk factors for tuberculosis infection emphasizes the importance of including tuberculosis in the differential diagnosis of spinal cord masses.

Option Hedging with Smooth Market Impact

2016 ◽  
Vol 02 (01) ◽  
pp. 1650002 ◽  
Author(s):  
Robert Almgren ◽  
Tianhui Michael Li
Keyword(s):  
Discrete Time ◽  
Market Impact ◽  
Large Collection ◽  
Public Markets ◽  
The Public ◽  
Option Hedging ◽  
A Value ◽  
First Case ◽  
Black Scholes ◽  
Optimal Hedge

We consider intraday hedging of an option position, for a large trader who experiences temporary and permanent market impact. We formulate the general model including overnight risk, and solve explicitly in two cases which we believe are representative. The first case is an option with approximately constant gamma: the optimal hedge trades smoothly towards the classical Black–Scholes delta, with trading intensity proportional to instantaneous mishedge and inversely proportional to illiquidity. The second case is an arbitrary non-linear option structure but with no permanent impact: the optimal hedge trades toward a value offset from the Black–Scholes delta. We estimate the effects produced on the public markets if a large collection of traders all hedge similar positions. We construct a stable hedge strategy with discrete time steps.

The relationship between the serum free light chain assay and serum immunofixation electrophoresis, and the definition of concordant and discordant free light chain ratios

Blood ◽  
2009 ◽  
Vol 114 (1) ◽  
pp. 38-39 ◽  
Author(s):  
Seema Singhal ◽  
Eric Vickrey ◽  
Jairam Krishnamurthy ◽  
Veerpal Singh ◽  
Sharon Allen ◽  
...  
Keyword(s):  
Light Chain ◽  
Normal Serum ◽  
Free Light Chain ◽  
Serum Free ◽  
Serum Free Light Chain ◽  
Immunofixation Electrophoresis ◽  
Serial Samples ◽  
Definition Of ◽  
The Relationship

Abstract“Stringent” complete remission in myeloma has been defined by a normal serum free light chain ratio (SFLCR) in addition to the standard criteria for CR. 2648 serial samples from 122 IgG or IgA myeloma patients were studied to explore the relationship between SFLCR and serum immunofixation electrophoresis (SIFE). SFLCR was normal in 34% of cases with positive SIFE and abnormal in 66%. SFLCR was normal in 69% of cases with negative SIFE and abnormal in 31%. When evaluated with SIFE as the benchmark, the sensitivity of SFLCR was 66% and specificity was 69%. These findings were unchanged when abnormal SFLCR values were classified as concordant (< 0.26 for λ disease and > 1.65 for κ) or discordant (< 0.26 for κ disease and > 1.65 for λ). Additional studies are required to determine the temporal relationship between SFLCR normalization and paraprotein clearance. Until then, the role of SFLCR in defining response remains controversial.

Profiling Rotors for Limaçon-to-Limaçon Compression-Expansion Machines

2005 ◽  
Vol 128 (4) ◽  
pp. 787-793 ◽  
Author(s):  
Ibrahim A. Sultan
Keyword(s):  
Nonlinear Equations ◽  
Volumetric Efficiency ◽  
Radial Clearance ◽  
A Value ◽  
First Case ◽  
Rotor Profile ◽  
Mathematical Expressions ◽  
Second Mode ◽  
Extreme Care

Limaçon-to-limaçon compression-expansion machines have housings and rotors whose profiles are manufactured of limaçon curves. For these machines to perform satisfactorily, extreme care should be given to the geometric particulars of their rotor profile. The main characteristics that govern the quality of the rotor profile are the volumetric efficiency and the prevention of interference. In this work, the interference problem is handled from two different mathematical standpoints: the slope of tangents to both the rotor and housing curves at the apices; and the value of the minimum radial clearance that separates the two limaçon curves. In the first case, mathematical expressions, relating the radii of the limaçon base circles is presented to ensure that interference would not take place during normal operations of the limaçon-to-limaçon machine. The second mode of analysis produces a set of nonlinear equations that can be solved to obtain a value of the radial clearance. This value has to be machined off the rotor profile to prevent interference. A numerical example is given at the end of the paper to prove the validity of the models proposed and graphs are produced to support the claims presented.

scholarly journals Lipoleiomyosarcoma an extremely unusual sarcoma of uterus: A case report

1970 ◽  
Vol 2 (1) ◽  
pp. 67-70 ◽  
Author(s):  
Abhimanyu Jha ◽  
Gita Sayami ◽  
Deepti Adhikari
Keyword(s):  
Smooth Muscle ◽  
Abdominal Pain ◽  
Case Report ◽  
Postmenopausal Woman ◽  
Unusual Case ◽  
Histopathological Examination ◽  
Abdominal Mass ◽  
Lower Abdominal Pain ◽  
First Case ◽  
Well Differentiated

Uterine lipoleiomyosarcoma is a heterologous sarcoma composed of variable proportions of malignant lipoblasts histologically corresponding to well differentiated liposarcoma and malignant smooth muscle cells corresponding to leiomyosarcoma. Finding of benign lipomatous component in a typical leiomyoma (lipoleiomyoma) is not an uncommon, however, lipoleiomyosarcoma is an extremely rare malignant tumor and only very few cases have been reported so far. We report a case of lipoleiomyosarcoma of uterine corpus in a postmenopausal woman presenting with lower abdominal pain and abdominal mass. Diagnosis of lipoleiomyosarcoma was confirmed by histopathological examination of hysterectomy specimen. This is the first case of lipoleiomyosarcoma of uterus reported from Nepal. Keywords: Lipoleiomyosarcoma, uterus, histopathology, unusual case.   doi:10.3126/njog.v2i1.1482    N. J. Obstet. Gynaecol Vol. 2, No. 1, p. 67 - 70 May -June 2007

Hyperphosphorylated paratarg-7: a new molecularly defined risk factor for monoclonal gammopathy of undetermined significance of the IgM type and Waldenström macroglobulinemia

Blood ◽  
2011 ◽  
Vol 117 (10) ◽  
pp. 2918-2923 ◽  
Author(s):  
Sandra Grass ◽  
Klaus-Dieter Preuss ◽  
Alexandra Wikowicz ◽  
Evangelos Terpos ◽  
Marita Ziepert ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Monoclonal Gammopathy ◽  
Immunoglobulin A ◽  
Cell Receptor ◽  
Carrier State ◽  
Waldenström Macroglobulinemia ◽  
Waldenstrom Macroglobulinemia ◽  
Phosphatase Treatment ◽  
Increased Risk ◽  
Undetermined Significance

Abstract We recently described paratarg-7 (P-7), a protein of unknown function, as the target of 15% of immunoglobulin A (IgA) and IgG paraproteins in monoclonal gammopathy of undetermined significance (MGUS) and multiple myeloma. To determine the frequency of P-7 as a paraprotein target in IgM-MGUS and Waldenström macroglobulinemia (WM), sera from patients with IgM-MGUS/WM were tested for reactivity with recombinant P-7 by enzyme-linked immunoabsorbent assay. The specificity of the paraprotein-mediated reaction was shown by absorption studies and cloning of the respective B-cell receptor. The paraproteins of 18 (9 WM and 9 IgM-MGUS) of 161 patients (11%) reacted with P-7. Isoelectric focusing and phosphatase treatment showed that P-7 was hyperphosphorylated (pP-7) in all patients with an anti–P-7-specific IgM paraprotein tested. Because only 4 of 200 healthy controls (2%) were carriers of pP-7, pP-7 carrier state is associated with a significantly increased risk (odds ratio = 6.2; P = .001) for developing IgM-MGUS/MW. Family analyses showed that the pP-7 carrier state is inherited as a dominant trait. After IgA/IgG-MGUS and multiple myeloma, IgM-MGUS/WM is the second neoplasia associated with pP-7 carrier state. The dominant inheritance of pP-7 explains cases of familial IgM-MGUS/WM and enables the identification of family members at increased risk.

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