Case report of osteolytic lesions in a patient with multisystem granulomatous disease

2021 ◽  
Vol 14 (6) ◽  
pp. e242685
Author(s):  
Henco Nel ◽  
Brad Davis ◽  
Brendan Adler ◽  
Eli Gabbay
Keyword(s):  
Cervical Lymphadenopathy ◽  
Granulomatous Inflammation ◽  
Initial Response ◽  
Response To Treatment ◽  
Caucasian Woman ◽  
Granulomatous Disease ◽  
Osteolytic Lesions ◽  
Moderate Growth ◽  
Systemic Corticosteroids ◽  
Lytic Lesions

We present a case of a 70-year-old Caucasian woman with multisystem granulomatous disease involving her lungs, bones and lymph nodes. The patient initially presented with cervical lymphadenopathy and subsequently developed progressive breathlessness. Imaging revealed extensive mediastinal, hilar and intra-abdominal lymphadenopathy as well as bilateral pulmonary parenchymal infiltrates. Lymph node and lung biopsy confirmed non-necrotising granulomatous inflammation while a BAL showed scanty growth of Cryptococcus neoformans and moderate growth of Staphylococcus aureus. The patient received intravenous ceftriaxone and had a good response to treatment. She also completed 3 months of oral fluconazole. Although a diagnosis of sarcoidosis was considered most likely, the patient was not initially started on systemic corticosteroids due to concern around possible infection and initial response to antimicrobials. However, her exercise tolerance gradually deteriorated. A craniofacial CT revealed multiple lytic lesions involving the skull and visualised cervical spine. Biopsy of a clivus lesion revealed non-necrotising granulomatous inflammation while fungal cultures and histopathological stains were negative. The patient was diagnosed with widespread sarcoidosis and she was initiated on prednisolone and methotrexate which led to marked clinical and radiological improvement.

scholarly journals Atypical Early Onset Juvenile Sarcoidosis Presenting with Osteolytic Lesions

2018 ◽  
Vol 1 (1) ◽  
pp. 1-4
Author(s):  
Samar Alwafi ◽  
Razan Alluhaibi ◽  
Sahar Alsharif ◽  
Bader AlOmair
Keyword(s):  
Early Onset ◽  
Pediatric Patients ◽  
Clinical Course ◽  
Age Of Onset ◽  
Granulomatous Disease ◽  
Initial Manifestation ◽  
Osteolytic Lesions ◽  
Lytic Lesions ◽  
Osseous Sarcoidosis ◽  
Juvenile Sarcoidosis

Sarcoidosis is a potentially fatal systemic inflammatory granulomatous disease, can occur in adult and pediatric patients, but it is relatively rare in children. Juvenile sarcoidosis has a diverse clinical course depending on the age of onset. Bone involvement is rarely noted in children with sarcoidosis and usually seen late in the course of the disease and is rarely the initial manifestation. Here we report a case of early onset juvenile sarcoidosis revealed by cutaneous and osseous lytic lesions of the phalanges. Keywords: Atypical, Early onset, Juvenile, Sarcoidosis, Osteolytic lesions, Osseous sarcoidosis, Phalanges.

scholarly journals The p47phox mouse knock-out model of chronic granulomatous disease.

1995 ◽  
Vol 182 (3) ◽  
pp. 751-758 ◽  
Author(s):  
S H Jackson ◽  
J I Gallin ◽  
S M Holland
Keyword(s):  
Nadph Oxidase ◽  
Chronic Granulomatous Disease ◽  
Fungal Infections ◽  
Critical Role ◽  
Granulomatous Inflammation ◽  
Mammalian Host ◽  
Granulomatous Disease ◽  
Knock Out ◽  
Phagocyte Nadph Oxidase ◽  
Life Threatening

Chronic granulomatous disease (CGD) is caused by a congenital defect in phagocyte reduced nicotinamide dinucleotide phosphate (NADPH) oxidase production of superoxide and related species. It is characterized by recurrent life-threatening bacterial and fungal infections and tissue granuloma formation. We have created a mouse model of CGD by targeted disruption of p47phox, one of the genes in which mutations cause human CGD. Identical to the case in human CGD, leukocytes from p47phox-/- mice produced no superoxide and killed staphylococci ineffectively. p47phox-/- mice developed lethal infections and granulomatous inflammation similar to those encountered in human CGD patients. This model mirrors human CGD and confirms a critical role for the phagocyte NADPH oxidase in mammalian host defense.

scholarly journals Whole-Body Low-Dose Multidetector-Row CT in Multiple Myeloma: Guidance in Performing, Observing, and Interpreting the Imaging Findings

Life ◽  
2021 ◽  
Vol 11 (12) ◽  
pp. 1320
Author(s):  
Antonio Pierro ◽  
Alessandro Posa ◽  
Costanzo Astore ◽  
Mariacarmela Sciandra ◽  
Alessandro Tanzilli ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Low Dose ◽  
Plasma Cells ◽  
Traditional Approach ◽  
Response To Treatment ◽  
Whole Body ◽  
Newly Diagnosed ◽  
Low Dose Ct ◽  
Lytic Lesions ◽  
Total Body

Multiple myeloma is a hematological malignancy of plasma cells usually detected due to various bone abnormalities on imaging and rare extraosseous abnormalities. The traditional approach for disease detection was based on plain radiographs, showing typical lytic lesions. Still, this technique has many limitations in terms of diagnosis and assessment of response to treatment. The new approach to assess osteolytic lesions in patients newly diagnosed with multiple myeloma is based on total-body low-dose CT. The purpose of this paper is to suggest a guide for radiologists in performing and evaluating a total-body low-dose CT in patients with multiple myeloma, both newly-diagnosed and in follow-up (pre and post treatment).

scholarly journals Plasma cell myeloma lytic lesions mimicking vanishing bone syndrome in a young patient

2019 ◽  
Vol 5 (4) ◽  
pp. 20190025
Author(s):  
Margaret Mwania ◽  
Naushad Karim ◽  
Sarah Wambui ◽  
Shamshudin Mohammedali ◽  
Allan Njau
Keyword(s):  
Bone Marrow ◽  
Plasma Cell ◽  
Plasma Cells ◽  
Appendicular Skeleton ◽  
Plasma Cell Myeloma ◽  
Osteolytic Lesions ◽  
The Past ◽  
Lytic Lesions ◽  
Hiv Seropositive ◽  
Bone Marrow Disorder

Plasma cell myeloma is a bone marrow disorder characterized by neoplastic proliferation of plasma cells within the bone marrow replacing normal cells. We present a case report of a 25-year-old female with bilateral lower and upper limb pains. She had been seen in various health facilities for the past 2 years with progressively worsening disability. Skeletal survey revealed multiple osteolytic lesions in the appendicular skeleton resembling vanishing bone syndrome. Ultrasound-guided biopsy was done with histological diagnosis of plasma cell myeloma. This case is unique because of the young age at presentation, HIV seropositive status and atypical appearance of the lesions.

Prediction of outcome in neurotic disorder: a 5-year prospective study

1998 ◽  
Vol 28 (5) ◽  
pp. 1149-1157 ◽  
Author(s):  
H. SEIVEWRIGHT ◽  
P. TYRER ◽  
T. JOHNSON
Keyword(s):  
Initial Response ◽  
Favourable Outcome ◽  
Dysthymic Disorder ◽  
Response To Treatment ◽  
Behaviour Therapy ◽  
Neurotic Disorder ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Predicting Outcome

Background. There have been no previous studies of the outcome of different neurotic disorders in which a prospective group with original randomization to treatment have been followed up over a long period. Such studies are important in identifying the factors associated with good and poor outcome.Methods. A 5-year follow-up assessment was made of a cohort of 210 psychiatric out-patients seen in general practice psychiatric clinics with a DSM-III diagnosis of generalized anxiety disorder (71), panic disorder (74) or dysthymic disorder (65) and randomized to drug treatment, cognitive and behaviour therapy, and self-help. A total of 182 of the patients (87%) were assessed after 5 years by examination of hospital and GP records using a standardized procedure and outcome determined with a four-point outcome scale.Results. One hundred and seven (60%) of the patients had a favourable outcome but the remainder continued to be handicapped either intermittently or continuously throughout the 5-year period. Analysis of the value of initial data in predicting outcome using polychotomous step-wise logistic regression revealed that five variables were significant predictors of poor prognosis: older age; recurrent episodes; the presence of personality disorder at entry; general neurotic syndrome at entry; and symptom severity after 10 weeks. The initial DSM diagnosis and original treatment given, together with ten other variables, were of no predictive value.Conclusions. The long-term outcome of neurotic disorder is better predicted by age, personality and recency of onset than by other clinical variables with the exception of initial response to treatment.

scholarly journals Chronic Granulomatous Disease: A Rare Hereditary Immuno-deficiency Disorder in a Young Boy with Its Adverse Consequences

2016 ◽  
Vol 33 (3) ◽  
pp. 156-160
Author(s):  
Mamunur Rashid ◽  
Asfia Nigar ◽  
Md Rashidul Hassan
Keyword(s):  
Chronic Granulomatous Disease ◽  
Granulomatous Inflammation ◽  
Primary Immunodeficiency Disease ◽  
Granulomatous Disease ◽  
Phagocytic Cells ◽  
X Ray ◽  
Chest X Ray ◽  
Immunodeficiency Disease ◽  
Bacteria And Fungi ◽  
Long Acting

Chronic granulomatous disease (CGD) is a primary immunodeficiency disease which results from absence of the NADPH oxidase in the professional phagocytic cells neutrophils, monocytes, macrophages and eosinophils. Deficiency of this oxidase renders the patient liable to infection by bacteria and fungi, and, as the name of the disease suggests, to chronic granulomatous inflammation. Here, a young boy presented with increasing breathlessness and productive cough had recurrent episode of pulmonary infection since his childhood. Repeated Chest X-ray and CT scan showed homogenous opacities at different places of lung in different occasions with bilateral reticulo-nodular opacities. Mulipleoraganisms were isolated from sputum at different times. A nitroblu-tetrazolium test (NBT) was done abroad which was positive and confirmed his diagnosis. Since then, He had prophylactic fluconazol and sulphamethoxazoltrimethoprime daily and pneumocaccal and influenza vaccination regularly and proper treatment of acute infective episodes accordingly. In spite all these measures, repeated infection caused grievous harm to his lung leading to irreversible pulmonary fibrosis and bronchiectasis. As a consequence, he became home bound, oxygen dependant and dependant on regular use of long acting bronchodilators in different form. Hematopoeitic stem cell transplantation was advised which was not affordable for his parents. Now, this young boy is waiting for further assaults to his lungs and further deterioration and ultimate hopeless outcomes.J Bangladesh Coll Phys Surg 2015; 33(3): 156-160

The long-term follow-up of treatment with corticosteroid injections in patients with carpal tunnel syndrome. When are multiple injections indicated?

2012 ◽  
Vol 38 (6) ◽  
pp. 634-639 ◽  
Author(s):  
M. Berger ◽  
M. Vermeulen ◽  
J. H. T. M. Koelman ◽  
I. N. van Schaik ◽  
Y. B. W. E. M. Roos
Keyword(s):  
Carpal Tunnel Syndrome ◽  
Carpal Tunnel ◽  
Corticosteroid Injection ◽  
Initial Response ◽  
Response To Treatment ◽  
Good Outcome ◽  
Tunnel Syndrome ◽  
Corticosteroid Injections

The objective of this prospective study was to investigate the long-term effect of one or more local corticosteroid injections in patients with carpal tunnel syndrome and whether a good response can be predicted. Follow-up visits took place at 3 weeks, 6 months, and 1 year after the first corticosteroid injection. Thirty of the 120 patients (25%) had a good outcome with a single injection, 11 additional patients (9%) needed a second injection, and five patients (4%) needed a third injection to reach a good outcome after 1 year. Of patients with an initial good treatment response, 28 (52%) had a good outcome after 1 year compared with 18 (27 %) who had an initially moderate or no response to treatment. One-third of patients with carpal tunnel syndrome had a long-term beneficial effect from corticosteroid injection, especially when they had a good initial response.

scholarly journals Sarcoidosis Presenting as Massive Splenic Infarction

2012 ◽  
Vol 2012 ◽  
pp. 1-2 ◽  
Author(s):  
Ishita Patel ◽  
Mediola Ismajli ◽  
Alan Steuer
Keyword(s):  
Granulomatous Inflammation ◽  
Splenic Infarction ◽  
Granulomatous Disease ◽  
Unknown Aetiology ◽  
Rare Occurrence ◽  
Massive Splenomegaly

Sarcoidosis is a multisystem granulomatous disease of unknown aetiology. Granulomatous inflammation involving the spleen is common and associated with splenomegaly. However, massive splenomegaly is a rare occurrence. Infrequently massive splenomegaly can result in splenic infarction. Massive splenic infarction in sarcoidosis has, to our knowledge, not been previously reported. We present a case of a woman presenting with massive splenic infarction and sarcoidosis confirmed by granulomatous inflammation of the liver.

scholarly journals A Case of Plasmacytoid Dendritic Cell Leukemia

2013 ◽  
Vol 59 (2) ◽  
pp. 111-114
Author(s):  
Judit Beáta Köpeczi ◽  
I Benedek ◽  
Erzsébet Benedek ◽  
Enikő Kakucs ◽  
Aliz Tunyogi ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Initial Response ◽  
Skin Lesions ◽  
High Rate ◽  
Response To Treatment ◽  
Cell Leukemia ◽  
Cutaneous Lesions ◽  
Hla Dr

AbstractIntroduction: Plasmacytoid dendritic cell leukemia is a rare subtype of acute leukemia, which has recently been established as a distinct pathologic entity that typically follows a highly aggressive clinical course in adults. The aim of this report is to present a case of plasmacytoid dendritic cell leukemia due to its rarity and difficulty to recognize and diagnose it.Case report: We present a case of a 67 year-old man who presented multiple subcutaneous lesions on his face, neck, chest and upper extremities with reddish-brown, brown colour. In the bone marrow aspirate 83% of the blast cells were found. Immunophenotypically the blasts were positive for CD4, CD56, CD123 (high intensity), CD36, CD22, CD10 (10.42%), CD33, HLA-DR, CD7 (9.24%), CD38 (34.8%) and negative for CD13, CD64, CD14, CD16, CD15, CD11b, CD11c, CD3, CD5, CD2, CD8, CD19, CD20, CD34. The skin biopsy showed lymphohistiocytoid infiltration in the dermis. The patient was diagnosed with acute plasmacytoid dendritic cell leukemia and received polychemotherapy with rapid response of skin lesions and blastic infiltration of the bone marrow. After 3 courses of polychemotherapy the cutaneous lesions reappeared and multiplied. The blast infiltration in the bone marrow increased to 70%. A more aggressive polychemotherapy regimen was administered, but the patient presented serious complications (febrile neutropenia) and died in septic shock 8 months after the initiation of treatment.Conclusions: Immunophenotyping of blasts cells is indispensable in the diagnosis of plasmacytoid dendritic cell leukemia. The CD4+, CD56+, lin-, CD123 ++high, CD11c-, CD36+, HLA-DR+, CD34-, CD45+ low profile is highly suggestive for pDCL. The outcome of plasmacytoid dendritic cell leukemia is poor. Despite the high rate of initial response to treatment, early relapses occur and the patients die of disease progression.

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