A Rare Case of Primary Amyloidosis, Presenting with Severe Pulmonary Hypertension and Bilateral Pleural Effusion.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 5100-5100
Author(s):  
Aref Agheli ◽  
Alka Arora ◽  
Maged Khalil ◽  
Theresa Dumlao ◽  
Seema Naik ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pleural Effusion ◽  
Basement Membranes ◽  
Bilateral Pleural Effusion ◽  
Response To Treatment ◽  
Primary Amyloidosis ◽  
X Rays ◽  
Severe Pulmonary Hypertension ◽  
Rare Presentation ◽  
History Of

Abstract Pulmonary arterial hypertension secondary to vascular deposition is a rare presentation of Primary Amyloidosis. The natural history of pulmonary hypertension and concomitant right-sided cardiac failure is not very well understood and is associated with a very short median survival. This syndrome is a marker of advanced Amyloidosis Persistent pleural effusion is another much rarer presentation of pulmonary involvement in Primary Amyloidosis and is secondary to pleural infiltration, which sometimes is difficult to distinguish from the cardiomyopathic causes. The incidence of pleural effusion in primary Amyloidosis is 1–2%. This syndrome also has a very poor prognosis, and has limited response to treatment, however, pleurodesis has been temporarily useful in some cases. Case report: A 74 year old African American female, non-smoker, with history of hypertension, who presented with shortness of breath and persistent productive cough and 18 pounds weight loss for the last six months. The initial chest x-rays showed bilateral pleural effusion and a transudate fluid on analysis. Cardiac workup for an atypical chest pain revealed moderate to severe pulmonary hypertension without myocardial infiltration on 2-D echocardiogram, and normal coronary arteries on cardiac catheterization. Extensive workup did not show any secondary cause of pulmonary hypertension, however, the patient gradually developed nephrotic range proteinuria and kidney biopsy showed infiltration of 10 nm fibrils, involving 3+ segmental mesangium, and 2+ segmental glomerular basement membranes. This was consistent with renal Primary Amyloidosis, AL Lambda type. A random urine and serum sample for immunofixation also revealed an M- spike of 23 mg/dl in gamma region. A concomitant bone marrow biopsy revealed presence of monoclonal IgG lambda plasma cell (CD38 bright) population, without any B-cell or T-cell markers. Conclusion: Pulmonary hypertension and pleural effusion are two rare and lethal presentations of pulmonary involvements in Primary Amyloidosis, and it should be considered in the differential diagnosis of any patient presenting with pulmonary symptoms. There is no definite treatment of these syndromes, although combinations of steroids and melphalan, along with colchicines, vincristine, adriamycin, and thalidomide have been used with minimum satisfactory outcomes. Autologous stem cell transplantation (SCT) provides the opportunity to give higher doses of chemotherapy and may be of benefit in some cases.

scholarly journals Pseudo-pseudo Meigs’ syndrome: a rare presentation of systemic lupus erythematosus

Reumatismo ◽  
2019 ◽  
Vol 71 (2) ◽  
pp. 108-112 ◽  
Author(s):  
G. Tansir ◽  
P. Kumar ◽  
A. Pius ◽  
S.K. Sunny ◽  
M. Soneja
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pleural Effusion ◽  
Autoimmune Disease ◽  
Lupus Erythematosus ◽  
Abdominal Distension ◽  
Ca 125 ◽  
Systemic Lupus ◽  
Rare Presentation ◽  
The Past ◽  
History Of

Systemic lupus erythematosus (SLE) is a chronic inflammatory multisystem autoimmune disease. Ascites when associated with pleural effusion and raised CA-125 levels in SLE patient, is known as pseudo-pseudo Meigs’ syndrome (PPMS). This is the case of a 22-year-old lady who presented with complaints of abdominal distension for one month and had a history of spontaneous abortion in the past. Abdominal imaging did not reveal any tumor and after extensive workup a diagnosis of PPMS was made. She was successfully treated with steroids, hydroxychloroquine and cyclophosphamide.

scholarly journals MULTIGRAVIDA WITH UNCONTROLLED HYPERTHYROID AND BILATERAL PLEURAL EFFUSION

2020 ◽  
Vol 4 (2) ◽  
pp. 198-203
Author(s):  
Muhammad Al Farisi Sutrisno ◽  
Herlambang Herlambang ◽  
Firmansyah Firmansyah
Keyword(s):  
Pleural Effusion ◽  
Fetal Heart ◽  
Gestational Hypertension ◽  
Thyroid Function Test ◽  
Bilateral Pleural Effusion ◽  
Thyroid Gland Function ◽  
Drug Of Choice ◽  
Function Test ◽  
History Of ◽  
Gland Function

BACKGROUND: Hyperthyroid is a hypermetabolic condition caused by abnormal thyroid gland function resulting in overproduction and overexpression of thyroid hormone. The prevalence of hyperthyroid during pregnancy is 0.1-0.4%, where 85% of case are presented as grave’s disease. OBJECTIVE: To report the treatment of uncontrolled hyperthyroid during pregnancy.METHOD: Case Report CASE:  Ms. S, Female, 33 years old, presenting with brethlessness since 5 days before admission. Breathlessness persist and aggravated by lying down position. The patient has history of hyperthyroid since 1 years before admission. The blood pressure was 120/80 mmHg, respiration rate 28 times/min, and body temperature 36,7oC. Uterus fundal height 26 cm, cephalic presentation, fetal heart rate 130 times/min, single fetus intrauterine and alive. Laboratoric test for leukocyte: 21,300/ul, T4 level 22.8 mg/dl dan T3 level 2.9 mg/dl. The patient diagnosed with G3P2A0 31-week gestational age single alive fetus intrauterine with uncontrolled hyperthyroid and bilateral pleural effusion. Treatment consist of propylthiouracil as the drug of choice for anti-thyroidal drug, nifedipine for gestational hypertension and furosemide to treat the pleural effusion.  CONCLUSION: History taking, physical examination, thyroid function test, and maintaining euthyroidism during pregnancy is a key to reduce the risk of maternal and fetal complication.KEYWORDS : hyperthyroid, pregnancy, IUGR

scholarly journals Yellow Nail Syndrome or Diffuse Lymphatic Network Disease

2002 ◽  
Vol 45 (4) ◽  
pp. 181-182 ◽  
Author(s):  
Konstantinos A Christu ◽  
Chaido Pastaka ◽  
Dimitrios Papadopoulos ◽  
Eleni Klimi ◽  
Konstantinos I Gourgoulianis
Keyword(s):  
Pleural Effusion ◽  
Bilateral Pleural Effusion ◽  
Intestinal Lymphangiectasia ◽  
Yellow Nail Syndrome ◽  
History Of ◽  
Lymphatic Network

We report a man aged 68 years old with pneumothorax and chronic bilateral pleural effusion in association with a history of yellow nails. The diagnosis of yellow nail syndrome based on yellow nails, lymphedema, chronic pleural effusion and intestinal lymphangiectasia.

scholarly journals RHEUMATOID ARTHRITIS 1ST TIME PRESENTING WITH BILATERAL PLEURAL EFFUSION- A RARE PRESENTATION

2019 ◽  
Vol 05 (05) ◽  
pp. 404-406
Author(s):  
Sayan Malakar ◽  
Kailash Nath Sharma ◽  
Abhinav Rana ◽  
Tarun Sharma
Keyword(s):  
Rheumatoid Arthritis ◽  
Pleural Effusion ◽  
Bilateral Pleural Effusion ◽  
Rare Presentation

Utility Of Pre-Chemotherapy Echocardiograms

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 2950-2950
Author(s):  
John Allen Steuter ◽  
Philip Bierman ◽  
R. Gregory Bociek ◽  
Martin Bast
Keyword(s):  
Pulmonary Hypertension ◽  
Cardiac Function ◽  
Chemotherapy Regimen ◽  
Systolic Pressure ◽  
Left Ventricular ◽  
Valvular Disease ◽  
Pulmonary Artery Systolic Pressure ◽  
Severe Pulmonary Hypertension ◽  
History Of ◽  
Cardiac Diagnosis

Abstract Anthracycline-based chemotherapy is widely used in a variety of regimens for Hodgkin’s Lymphoma (HL) and Non-Hodgkin’s Lymphoma (NHL). Unfortunately this agent is associated with cardiotoxicity, especially in larger cumulative doses. Doxorubicin cardiotoxicity is characterized by a dose-dependent decline in mitochondrial oxidative phosphorylation. Reactive oxygen species, generated by the interaction of doxorubicin with iron, can then damage the myocytes causing myofibrillar loss, cytoplasmic vacuolization, and apoptosis. Current NCCN guidelines for HL and NHL state that left ventricle function is recommended in patients who will receive such chemotherapy. Subsequent evaluations are recommended at a cumulative dose of doxorubicin of at least 300 mg/m² and periodically thereafter during the course of therapy. Despite these recommendations, there is little literature about the usefulness of this approach, the rate of abnormal findings, and whether routine testing changes management. A list of patients with HL or NHL treated with anthracycline-based chemotherapy from the University of Nebraska Medical Center from August 2004-May 2012 was obtained from our Lymphoma Registry. Baseline characteristics of age, disease diagnosis, gender, and prior history of cardiac diagnosis were collected. Prior cardiac diagnosis specified as patients with a past history of atrial fibrillation/flutter, supraventricular tachycardia, heart block requiring pacemaker implantation, coronary artery disease, valvular disease, pulmonary hypertension, or cardiomyopathy. Charts were reviewed for pre-chemotherapy evaluation of left ventricular function and the method of evaluation (echocardiogram, nuclear, cardiac MRI were included). Additionally, post treatment evaluation of cardiac function was evaluated for any change. The individual therapy for each patient was reviewed to determine if findings from the cardiac evaluation modified treatment regimens. A left ventricular function less than 50% was considered abnormal. Echocardiogram findings of moderate to severe valvular disease, diastolic dysfunction (grade 1-3), and moderate or severe pulmonary hypertension (moderate = pulmonary artery systolic pressure of 45-60, severe = pulmonary artery systolic pressure of > 60) were collected. We identified 309 patients from the UNMC lymphoma database. Of these 219 (71%) had an echocardiogram performed prior to therapy and documented in the patient record. Their mean age was 54.2 years of age with 53% of them being male and 47% female. 188 of the 219 (86%) had no prior cardiac diagnosis as defined previously. From this group 22 of the 188 (10%) had a pre-chemotherapy echocardiogram that demonstrated one of the following: moderate-severe valvular disease, diastolic dysfunction, moderate-severe pulmonary hypertension or abnormal EF (<50%). However, none of these findings altered the chemotherapy regimen for the 22 patients. 31 of the 219 patients with echocardiograms carried a prior cardiac diagnosis. 4 of the 31 had an alteration in their chemotherapy regimen as a result. Post therapy echocardiograms showed no change in cardiac function. 5 of the 219 patients were diagnosed with adriamycin induced cardiomyopathy following treatment. All 5 patients had no prior cardiac history and pre-chemotherapy echocardiograms were normal. These finding suggest that current methods of evaluating cardiac function prior to chemotherapy and risk stratifying patients are inadequate and do not alter patient outcomes. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Malignant melanoma presenting as an isolated pleural effusion

2015 ◽  
Vol 75 (2) ◽  
Author(s):  
M. Shameem ◽  
J. Akhtar ◽  
U. Baneen ◽  
N. Ahmad Khan ◽  
R. Bhargava ◽  
...  
Keyword(s):  
Pleural Effusion ◽  
Malignant Melanoma ◽  
Pleural Fluid ◽  
Past History ◽  
Mediastinal Lymphadenopathy ◽  
Rare Presentation ◽  
Pigmented Lesion ◽  
S 100 ◽  
History Of ◽  
Enhanced Computed Tomography

Isolated pleural effusion is a very rare presentation of malignant melanoma. A 46 year-old male patient presented to us with complaints of shortness of breath during the previous month. A contrast enhanced computed tomography (CECT) imaging scan of his thorax showed right-sided pleural effusion with the absence of any mass lesion or mediastinal lymphadenopathy. Cytology of his pleural fluid showed pigmented cells suggestive of malignant melanoma. Staining of the pleural fluid cells with immunohistological markers for melanoma (HMB 45 and S 100) were positive. An examination of his skin did not reveal any pigmented lesion, nor was there a past history of malignant melanoma for this patient. The patient responded well to therapy for malignant melanoma and has remained asymptomatic for the last year. This patient was diagnosed with a rare case of primary malignant melanoma of the pleura presenting as isolated pleural effusion.

scholarly journals Pseudo-Meigs syndrome: a rare presentation of pedunculated fibroid

2020 ◽  
Vol 9 (7) ◽  
pp. 3052
Author(s):  
Sunita Dubey ◽  
Poonam Goel ◽  
Nidhi Pandey ◽  
Pavithra H. N.
Keyword(s):  
Pleural Effusion ◽  
Abdominal Distension ◽  
Uterine Sarcoma ◽  
Ca 125 ◽  
Rare Presentation ◽  
Atypical Symptoms ◽  
Extensive Evaluation ◽  
History Of ◽  
Intramural Fibroids ◽  
Rule Out

Serosal fibroid of uterus are usually asymptomatic but rarely; it may present with atypical symptoms to simulate malignancy and needs extensive evaluation. Authors are reporting a case of 26 years old P1L1 female with history of asymptomatic multiple intramural fibroids since 6 years came with complained of progressively increasing abdominal distension and mild pain abdomen from 2-3 months. On evaluation, she had ascites, pleural effusion and raised Ca-125. MR imaging of pelvis revealed moderate ascites and pedunculated serosal fibroid in addition to intramural fibroids with normal bilateral ovaries. She was evaluated to rule out uterine sarcoma and tuberculosis but diagnosis of them could not be established. Finally, conclusion of Pseudo-Meigs syndrome was made. Myomectomy of single pedunculated fibroid relieved her symptoms. Though, subserosal fibroids are benign in pathology, timely surgery is must to avoid morbidity and mortality owing to massive ascites and pleural effusion.

scholarly journals P1728 Pulmonary hypertension in a pregnant Women - a rare anatomical aetiology

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
J Grade Santos ◽  
F Ferreira ◽  
M Loureiro ◽  
A Almeida ◽  
A Pereira ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Pulmonary Artery Pressure ◽  
Medical Therapy ◽  
Cardiac Mri ◽  
Left Pulmonary Artery ◽  
Severe Pulmonary Hypertension ◽  
Right Pulmonary Artery ◽  
History Of

Abstract A 32 year old female patient, with a medical history of an ill-characterized Pulmonary Arterial Hypertension associated with congenital heart disease, lost in the follow up with no medical therapy, attended an emergency department for a gynecological hemorrhage at 16 weeks of pregnancy. Due to high maternity mortality risk, informed consent was obtained, and termination of pregnancy was performed. She was then referred to our pulmonary hypertension center. At our center she had complains of fatigue with moderate intensity exertion, classified in a class II of the World Health Organization (WHO) classification, but was otherwise asymptomatic, with no history of dyspnea, angina or syncope. There was allusion to a self-limited episode of hemoptysis in the past. On physical examination she had an increased pulmonary component of the second heart sound, continuous heart murmur in left sternal border and no cyanosis (O2 peripheral saturation in the upper and lower limbs of 99% at room air). The performed echocardiograms (both transthoracic and transesophageal) showed an estimated systolic pulmonary artery pressure of 120 mmHg with severe right ventricular hypertrophy and systolic dysfunction. There was dilatation of the trunk and right pulmonary artery. The left pulmonary artery was not seen. Biochemical evaluation and viral serologies were unremarkable. The pulmonary function tests and the arterial blood gases were normal. Cardiac MRI demonstrated the presence of a right aortic arch and a right patent arterial duct. An anomalous origin of the left pulmonary artery from the ascending thoracic aorta could be noted. Associated congenital cardiac defects were excluded. A right heart catheterization confirmed the presence of severe pulmonary hypertension with mean pulmonary artery pressure of 86 mmHg and Pulmonary vascular resistance of 11 Wood Units. A large persistent arterial duct to the right pulmonary artery was confirmed with persistent left to right shunt. The left pulmonary artery was visualized when injection was performed in the aortic root. Coronary arteries were normally implanted. The patient was started on Sildenafil and Bosentan (later replaced by Macitentan due to hepatic toxicity). After 3 years of follow up, there was an improvement in symptoms and in the 6 minutes walking test, remaining in a low risk category and on a WHO class I. This case reports a very rare congenital abnormality identified in an adult patient. Despite the complex anatomy and severe pulmonary hypertension, the patient is reasonably well under medical therapy and close follow up. Abstract P1728 Figure. Cardiac MRI Cine Sequences

scholarly journals Severe pulmonary hypertension associated with hepatic arteriovenous malformation in a patient with hereditary haemorrhagic telangiectasia

2018 ◽  
pp. bcr-2018-226067
Author(s):  
Sakolwat Montrivade ◽  
Patinya Maneesow ◽  
Thamonwan Osotthanakorn ◽  
Pairoj Chattranukulchai
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Physical Examination ◽  
Arteriovenous Malformation ◽  
Asian Woman ◽  
Hereditary Haemorrhagic Telangiectasia ◽  
Severe Pulmonary Hypertension ◽  
Recurrent Epistaxis ◽  
History Of ◽  
Hepatic Arteriovenous Malformation

We report a case of 46-year-old Asian woman with a history of recurrent epistaxis who presented with dyspnoea on exertion. Physical examination revealed mucocutaneous telangiectasias and signs of heart failure. Further evaluation showed huge hepatic arteriovenous malformation and severe pulmonary hypertension. This case demonstrates an uncommon manifestation of hereditary haemorrhagic telangiectasia presented with severe pulmonary hypertension.

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