Leukemization of Follicular Lymphoma: Diagnostic Features and Clinical Course of a Rare Form of the Disease

Abstract Follicular lymphoma is an indolent lymphoma characterized by the (14;18) translocation, which leads to aberrant expression of Bcl-2. Translocations involving 8q24 are most commonly associated with Burkitt lymphoma and result in c-Myc overexpression. We report a case of follicular lymphoma of predominant small cleaved-cell type (grade 1) associated with both a t(14;18)(q32;q21) and a t(8;22)(q24;q11). The 8q24 translocation predicted an aggressive clinical course, as the lymphoma transformed into acute lymphoblastic leukemia within a year of initial diagnosis. Routine cytogenetic analysis is recommended at initial diagnosis of follicular lymphoma to better identify abnormalities that may predict prognosis and influence therapy.

Download Full-text

Clinical Course of Unusual Malignant Sarcomas of Head and Neck

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348947908800407 ◽

1979 ◽

Vol 88 (4) ◽

pp. 486-494 ◽

Cited By ~ 11

Author(s):

Michael Setzen ◽

Steven Sobol ◽

James M. Toomey

Keyword(s):

Head And Neck ◽

Recent Literature ◽

Clinical Course ◽

Clinical Manifestations ◽

Distinct Group ◽

Host Factors ◽

Sex Distribution ◽

Diagnostic Features ◽

Diagnostic Characteristics ◽

Therapeutic Responses

The clinical manifestations of 29 recently encountered sarcomas of the head and neck were analyzed in an attempt to define more accurately the diagnostic characteristics and therapeutic responses of these unusual tumors. The host factors of age, sex distribution, race, habits and associated features differ sufficiently to distinguish the sarcoma from the carcinoma population. In addition, the clinical course of sarcoma patients as monitored by mode of presentation, site of involvement, tumor histology, diagnostic features and certain elements of management and outcome, further indicates that these tumors can be defined and managed as a distinct group of lesions. A review of the recent literature supports the major conclusions of this study.

Download Full-text

A Case of Fulminant Hepatitis due to Echovirus 9 in a Patient on Maintenance Rituximab Therapy for Follicular Lymphoma

Case Reports in Hematology ◽

10.1155/2015/454890 ◽

2015 ◽

Vol 2015 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Ceri Morgan ◽

S. J. Thomson ◽

Joanne Legg ◽

Santosh Narat

Keyword(s):

Follicular Lymphoma ◽

Viral Infections ◽

Clinical Course ◽

Enterovirus Infection ◽

Risk Of Infection ◽

Unique Case ◽

Missed Opportunities ◽

Appropriate Treatment ◽

Increased Risk ◽

Viral Testing

Rituximab is a CD20 monoclonal antibody commonly used in the treatment of haematological malignancies. It causes lymphopenia with subsequent compromised humoral immunity resulting in an increased risk of infection. A number of infections and viral reactivations have been described as complicating Rituximab therapy. We report an apparently unique case of echovirus 9 (an enterovirus) infection causing an acute hepatitis and significant morbidity in an adult patient on maintenance treatment of Rituximab for follicular lymphoma. We also describe potential missed opportunities to employ more robust screening for viral infections which may have prevented delays in the appropriate treatment and thus may have altered the patient’s clinical course. We also make suggestions for lowering the threshold of viral testing in similar patients in the future.

Download Full-text

Family hyperaldosteronism type I: a clinical case and review of literature

Terapevticheskii arkhiv ◽

10.26442/terarkh2018909115-122 ◽

2018 ◽

Vol 90 (9) ◽

pp. 115-122

Author(s):

N M Chikladze ◽

O O Favorova ◽

I E Chazova

Keyword(s):

Adrenocorticotropic Hormone ◽

Clinical Case ◽

Clinical Course ◽

Chimeric Gene ◽

Monogenic Disease ◽

Type I ◽

Review Of Literature ◽

Aldosterone Secretion ◽

Rare Form ◽

Cerebrovascular Complications

Family hyperaldosteronism type I (glucocorticoids-remediable hyperaldosteronism) is a rare form of symptomatic arterial hypertension (AH), which often leads to the development of cerebrovascular complications. The disease is caused by the formation of the chimeric gene CYP11B2/CYP11B1. Expression of the chimeric gene is regulated by adrenocorticotropic hormone, and glucocorticoid therapy leads to a decrease in aldosterone secretion and normalization of blood pressure. The article presents the first clinical case of this monogenic disease diagnosed by us in Russia. The features of clinical course and treatment of the patient have been traced in the dynamics for 40 years of observation. Modern approaches to the diagnosis and treatment of this rare family form of hypertension are discussed.

Download Full-text

Acute paraplegia by spinal angiolipoma. Case report and literature review

Coluna/Columna ◽

10.1590/s1808-18512014130200403 ◽

2014 ◽

Vol 13 (2) ◽

pp. 147-149 ◽

Cited By ~ 2

Author(s):

Enrique Gonzalo Bovier ◽

Emiliano Gardino ◽

Lucas Nicolás Chemes ◽

Federico Nogueira ◽

Verónica Diana Romero

Keyword(s):

Benign Tumor ◽

Clinical Course ◽

Good Prognosis ◽

Rare Form ◽

Neurologic Recovery ◽

Compression Syndrome ◽

Spinal Compression ◽

Acute Paraplegia ◽

Intratumoral Bleeding ◽

Spinal Epidural

Spinal angiolipoma is an uncommon form of benign tumor. There are 142 reported cases and only one of acute paraplegia. We describe a case of a 39 year old male with acute spinal paraplegia angiolipoma in T4-T5 with surgical resolution three weeks after the onset of the neurological syndrome. Postoperative neurologic recovery was complete. The angiolipoma consists of mature fat cells and abnormal blood vessels. There are 2 types: non-infiltrating and infiltrating. Its clinical course is slow and progressive, it can be accelerated by vascular phenomena, intratumoral abscess, and pregnancy. There is just one report of spontaneous intratumoral hemorrhage and acute paraplegia, coinciding with our case. There is no consensus as to treatment, and release and complete resection are suggested, as it is a disease with good prognosis. Angiolipoma spinal epidural is a rare form of benign tumor, and the treatment of this pathology continues to be release and resection of the tumor, with a favorable prognosis, despite the delay in surgery, as in the case reported. Intratumoral bleeding should be considered a cause of acute spinal compression syndrome, as ocurred in our patient.

Download Full-text

Cutaneous Polyarteritis Nodosa (cPAN) in ab 5 Years Old Girl

COJ Nursing & Healthcare ◽

10.31031/cojnh.2018.02.000548 ◽

2018 ◽

Vol 2 (5) ◽

Author(s):

Lubna Nazir

Keyword(s):

Case Report ◽

Skin Biopsy ◽

Polyarteritis Nodosa ◽

Clinical Course ◽

Systemic Vasculitis ◽

Separate Entity ◽

Rare Form ◽

Cutaneous Polyarteritis Nodosa ◽

True Incidence ◽

Hands And Feet

Polyarteritis nodosa is a rare form of systemic vasculitis. Cutaneous polyarteritis nodosa (cPAN) is subtype of systemic PAN which predominantly affects the skin, extra-cutaneous findings include fever, malaise, myalgias, arthralgias, and neuropathy. The true incidence of cutaneous PAN is unknown. cPAN is recognized as a separate entity and is essentially a benign disorder which should be distinguished from systemic PAN, as the clinical course and management of the two conditions is different. In this case report, we have described a patient with fever and gangrene of both hands and feet. The patient underwent a very extensive workup in order to determine the cause of her condition, which finally turned out cPAN on skin biopsy

Download Full-text

Unbalanced Expression of Bcl-2 Family Proteins in Follicular Lymphoma: Contribution of CD40 Signaling in Promoting Survival

Blood ◽

10.1182/blood.v91.1.244 ◽

1998 ◽

Vol 91 (1) ◽

pp. 244-251 ◽

Cited By ~ 89

Author(s):

Paolo Ghia ◽

Vassiliki A. Boussiotis ◽

Joachim L. Schultze ◽

Angelo A. Cardoso ◽

David M. Dorfman ◽

...

Keyword(s):

B Cells ◽

Follicular Lymphoma ◽

Germinal Center ◽

Conventional Treatment ◽

Clinical Course ◽

Critical Role ◽

Relative Resistance ◽

Constitutive Overexpression

Abstract Although highly responsive, advanced stage follicular lymphoma (FL) is not curable with conventional treatment. This relative resistance is thought to be due to the t(14;18) that results in the constitutive overexpression of the death-inhibiting protein bcl-2. However, the observation that FL cells are sensitive to treatment in vivo and prone to apoptosis on in vitro culture questions whether bcl-2 alone is responsible for the pathogenesis and clinical behavior of this disease. Therefore, multiple genes are likely to be involved in both the lymphomagenesis and the clinical course of FL. We examined whether expression of other bcl-2 family genes might also be operative. Here, we show that FL cells display a different pattern of expression of bcl-2 family proteins from normal germinal center (GC) B cells that are thought to be their normal counterpart. FL cells express the death-suppressor proteins bcl-2, bcl-xL, and mcl-1; whereas GC B cells express bcl-xL and mcl-1 but also the proapoptotic proteins bax-α and bad. Although maintaining constitutive levels of bcl-2 and mcl-1, FL cells are not protected from apoptosis when cultured in vitro. Their propensity to undergo apoptosis is temporally associated with downregulation of bcl-xL. More importantly, activation of FL cells via CD40 not only prevents downregulation but increases the level of bcl-xL expression and results in promotion of survival. These results support the hypothesis that the overexpression of bcl-2 is not the only antiapoptotic mechanism responsible for the pathogenesis of FL. Survival of FL cells is determined by a number of death-inhibiting proteins, among which bcl-xL appears to have the most critical role. Moreover, these findings are consistent with the hypothesis that, although FL cells are malignant, they respond to microenvironmental signals such as CD40L that appear to contribute to their survival through the upregulation of death-inhibiting proteins.

Download Full-text

Molecular Profiling: A Case ofZBTB16-RARAAcute Promyelocytic Leukemia

Case Reports in Hematology ◽

10.1155/2017/7657393 ◽

2017 ◽

Vol 2017 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Stephen E. Langabeer ◽

Lisa Preston ◽

Johanna Kelly ◽

Matt Goodyer ◽

Ezzat Elhassadi ◽

...

Keyword(s):

Retinoic Acid ◽

Clinical Course ◽

Molecular Profiling ◽

Promyelocytic Leukemia ◽

Fusion Product ◽

Rare Form ◽

Trans Retinoic Acid ◽

All Trans Retinoic Acid ◽

Generation Sequencing

Several variantRARAtranslocations have been reported in acute promyelocytic leukemia (APL) of which the t(11;17)(q23;q21), which results in aZBTB16-RARAfusion, is the most widely identified and is largely resistant to therapy with all-trans retinoic acid (ATRA). The clinical course together with the cytogenetic and molecular characterization of a case of ATRA-unresponsiveZBTB16-RARAAPL is described. Additional mutations potentially cooperating with the translocation fusion product in leukemogenesis have been hitherto unreported inZBTB16-RARAAPL and were sought by application of a next-generation sequencing approach to detect those recurrently found in myeloid malignancies. This technique identified a solitary, low level mutation in theCEBPAgene. Molecular profiling of additional mutations may provide a platform to individualise therapeutic management in patients with this rare form of APL.

Download Full-text

RNA viruses: clinical and diagnostic features and principles of treatment

Terapevt (General Physician) ◽

10.33920/med-12-2004-09 ◽

2020 ◽

pp. 66-73

Author(s):

E. Levkova ◽

S. Savin ◽

K. Gulieva

Keyword(s):

Clinical Course ◽

Rna Viruses ◽

Diagnostic Algorithm ◽

Diagnostic Features ◽

Principles Of Treatment

The review presents generalized data on respiratory RNA viruses, features of clinical course and diagnostic algorithm. Special attention is paid to the principles oftreatment of diseases caused by RNA viruses.

Download Full-text

Genetic Abnormalities Involved in the Development and Progression of Follicular Lymphoma.

Blood ◽

10.1182/blood.v112.11.2049.2049 ◽

2008 ◽

Vol 112 (11) ◽

pp. 2049-2049

Author(s):

Karen E Deffenbacher ◽

George Wright ◽

Javeed Iqbal ◽

Huimin Geng ◽

Derville O’Shea ◽

...

Keyword(s):

Gene Expression ◽

High Resolution ◽

Follicular Lymphoma ◽

Copy Number ◽

Clinical Course ◽

B Cell Lymphoma ◽

Genetic Alterations ◽

Comparative Genomic ◽

Oncogenic Pathways ◽

Insight Into

Abstract Background: Follicular lymphoma (FL) is the most common indolent B-cell lymphoma and remains incurable by current therapeutic approaches. Clinical course is variable, and transformation into an aggressive lymphoma (t-FL) with marked worsening of prognosis occurs in 20–60% of patients. While Bcl2 gene translocation is a critical initiating event in the majority of FL cases, evidence indicates it is not sufficient for the development of a FL. Characterization of the genetic alterations subsequent to Bcl2 translocation will lend insight into the oncogenic pathways that contribute to FL pathogenesis and the molecular mechanisms underlying variability in clinical course. Methods: To define recurrent genomic copy number alterations (CNA) in FL, we performed high resolution array comparative genomic hybridization (aCGH) using the Affymetrix 500K SNP array platform. aCGH data were generated on a series of 112 FL cases with available gene expression profiling (GEP) and clinical information. Gene expression data were correlated with copy number data using the Gene Expression and Dosage Integrator (GEDI) algorithm developed at the NCI. Results: Selecting for abnormalities occurring in >10% of cases, the minimal common region (MCR) for 38 losses and 31 gains were defined. Novel common regions included gains on 15q11, 16p11, 5p14 and 19q13, and losses on 3q29, and 16p13. The MCR identified by aCGH were also compared with our existing cytogenetic data on 360 FL cases. MCR residing within the most frequent cytogenetic imbalances (>5%) were selected for analysis at the gene level to further refine these regions. These include gains on 1q21, 2p16, 7q11, 8q24, 12q13, 17q21, 18q21, 21q11, and X, and losses on 1p36, 6q, 10q, 13q34, and 17p13. Recurrent amplifications were detected for the 2p16, 15q11, and 17q21 MCR, while frequent uniparental disomy (UPD) was found to overlap the region of loss on 1p36. Recurrent UPD was also noted on 6p, 12q, 15q and 16p. For the majority of selected MCR, global expression of the genes residing in the MCR demonstrated an association with copy number status. Within these abnormalities, individual genes showing significant correlation with copy number were also identified. Conclusion: The combination of high resolution aCGH and GEP facilitated the identification of functionally relevant genes within the chromosomal abnormalities in FL. Delineation of these molecular targets will provide insight into the oncogenic pathways that contribute to FL disease pathogenesis and may provide novel therapeutic targets.

Download Full-text