scholarly journals Intravascular lymphomatosis presenting as skin lesions and subacute encephalopathy

2015 ◽  
Vol 53 (3) ◽  
pp. 273-281
Author(s):  
Inimioara Mihaela Cojocaru ◽  
Adina Mitru ◽  
Dana Nica ◽  
Carmen Maria Ardeleanu

Abstract Intravascular lymphomatosis is a neoplastic multisystemic disease; it is a rare subtype of diffuse large cell lymphoma characterized by the presence of lymphoma cells in the lumina of small vessels. A 49-year-old Caucasian woman was admitted to the Department of Internal Medicine for fatigue, night sweats, loss of weight, and multiple nodules in the forearms. Three months ago the patient’s family noticed problems with her cognitive function, she displayed difficulties with common daily tasks. The neurological examination revealed bradypsychia. Laboratory data showed modestly high levels of lactate dehydrogenase, and C-reactive protein. The day after admission, the patient had headache which raised in intensity; his mental status deteriorated, she was disoriented to time and place. She presented nucal rigidity. The CSF examination revealed a hemorrhagic aspect, elements 30/mm3, cytology: lymphocytes 90%, numerous erythrocytes, proteinorachia 96 mg/dL, glycorrachia 60 mg/dL. Intravenous Methylprednisolone (0.5 g two times a day) and Mannitol 20% 1g/kgw/day were administered for five days without response. She became comatose and she died six days after hospitalization. The post-mortem macroscopical brain examination showed a swallen brain, with diffuse hemorrhagic areas in the supratentorial subcortical regions. Microscopical examination showed capillaries, venules, and many arterioles distended by large malignant cells suggesting malignant lymphocytes which were intraluminal. Every organ was involved, except for bone marrow and lymph nodes. Immunohistochemical studies showed intensive staining for B cells and negative staining for factor VIII related antigen, a specific endothelial cell marker. Intravascular lymphomatosis was the post-mortem diagnostic. It represents a difficult diagnostic challenge which involves laboratory, imagistic and immunohistochemical investigations.

Healthcare ◽  
2021 ◽  
Vol 9 (2) ◽  
pp. 135
Author(s):  
Antonello Sica ◽  
Caterina Sagnelli ◽  
Beniamino Casale ◽  
Gino Svanera ◽  
Massimiliano Creta ◽  
...  

Background: The t (2; 5) chromosomal rearrangement of the ALK gene with nucleophosmin 1 gene (NPM1), resulting in an NPM1–ALK fusion, was first demonstrated in 1994 in anaplastic large cell lymphoma, (ALCL), a T-cell lymphoma responsive to cyclophosphamide, abriblastine, vincristine and prednisone in approximately 80% of cases; refractory cases usually respond favorably to brentuximab vedotin. These treatments are regarded as a bridge to allogeneic hematopoietic stem cell transplantation (allo-SCT). Nowadays, transplant procedures and the monitoring of chemotherapy patients proceed very slowly because the SARS-CoV-2 pandemic has heavily clogged the hospitals in all countries. Results: A 40-year-old Caucasian woman was first seen at our clinical center in June 2020. She had ALCL ALK+, a history of failure to two previous therapeutic lines and was in complete remission after 12 courses of brentuximab, still pending allo-SCT after two failed donor selections. Facing a new therapeutic failure, we requested and obtained authorization from the Italian drug regulatory agency to administer 250 mg of crizotinib twice a day, a drug incomprehensibly not registered for ALCL ALK +. Conclusions: The response to crizotinib was optimal since no adverse event occurred, and CT-PET scans persisted negative; this drug has proved to be a valid bridge to allo-SCT.


Blood ◽  
2003 ◽  
Vol 102 (6) ◽  
pp. 2213-2219 ◽  
Author(s):  
Marcel W. Bekkenk ◽  
Maarten H. Vermeer ◽  
Patty M. Jansen ◽  
Ariënne M. W. van Marion ◽  
Marijke R. Canninga-van Dijk ◽  
...  

Abstract In the present study the clinicopathologic and immunophenotypic features of 82 patients with a CD30– peripheral T-cell lymphoma, unspecified, presenting in the skin were evaluated. The purpose of this study was to find out whether subdivision of these lymphomas on the basis of cell size, phenotype, or presentation with only skin lesions is clinically relevant. The study group included 46 primary cutaneous CD30– large cell lymphomas and 17 small/medium-sized T-cell lymphomas as well as 17 peripheral T-cell lymphomas with both skin and extracutaneous disease at the time of diagnosis. Patients with primary cutaneous small- or medium-sized T-cell lymphomas had a significantly better prognosis (5-year-overall survival, 45%) than patients with primary cutaneous CD30– large T-cell lymphomas (12%) and patients presenting with concurrent extracutaneous disease (12%). The favorable prognosis in this group with primary cutaneous small- or medium-sized T-cell lymphomas was particularly found in patients presenting with localized skin lesions expressing a CD3+CD4+CD8– phenotype. In the primary cutaneous T-cell lymphoma (CTCL) group and in the concurrent group, neither extent of skin lesions nor phenotype had any effect on survival. Our results indicate that peripheral T-cell lymphomas, unspecified, presenting in the skin have an unfavorable prognosis, irrespective of the presence or absence of extracutaneous disease at the time of diagnosis, cell size, and expression of a CD4+ or CD8+ phenotype. The only exception was a group of primary cutaneous small- or medium-sized pleomorphic CTCLs with a CD3+CD4+CD8– phenotype and presenting with localized skin lesions.


2021 ◽  
Vol 27 ◽  
Author(s):  
L. M. Mikhaleva ◽  
A. L. Cherniaev ◽  
M. V. Samsonova ◽  
O. V. Zayratyants ◽  
L. V. Kakturskiy ◽  
...  

Background: Autopsies on COVID-19 deceased patients have many limitations due to necessary epidemiologic and preventative measures. The ongoing pandemic has caused a significant strain on healthcare systems and is being extensively studied around the world. Clinical data does not always corelate with post-mortem findings. The goal of our study was to find pathognomonic factors associated with COVID-19 mortality in 100 post-mortem full body autopsies.Materials and Methods: Following necessary safety protocol, we performed 100 autopsies on patients who were diagnosed with COVID-19 related death. The macroscopic and microscopic pathologies were evaluated along with clinical and laboratory findings.Results: Extensive coagulopathic changes are seen throughout the bodies of diseased patients. Diffuse alveolar damage is pathognomonic of COVID-19 viral pneumonia, and is the leading cause of lethal outcome in younger patients. Extrapulmonary pathology is predominantly seen in the liver and spleen. Intravascular thrombosis is often widespread and signs of septic shock are often present.Conclusion: The described pathological manifestations of COVID-19 in deceased patients are an insight into the main mechanisms of SARS-CoV-2 associated lethal outcome. The disease bears no obvious bias in severity, but seems to be more severe in some patients, hinting at genetic or epigenetic factors at play.


2003 ◽  
Vol 131 (7-8) ◽  
pp. 325-328 ◽  
Author(s):  
Jasmina Jovic ◽  
Marko Ercegovac ◽  
Branko Dozic ◽  
Dubravka Cvetkovic ◽  
Miroslava Zivkovic ◽  
...  

Intravascular lymphomatosis is an uncommon lymphoproliferative disorder characterised by intravascular distribution of neoplastic lymphoid cells (B- or T- lymphocites), what leads to occlusion of small vessels and causes signs and simptoms of disorder. Its initial predilection sites are the brain or the skin, and hematopoetic organs are usually spared. The signs and symptoms of the disorder are attributed to vascular occlusion. Clinical course is fatal, besides therapy. In our case disorder has started with partial epileptic seizures with secondary generalisation, and after that with motor aphasia, right hemi paresis, urinary incontinence and cognitive changes. She was hospitalized for several times, completely investigated, but with no conclusion. Seven months after appearance of symptoms, she died. And diagnosis was determined at autopsy. A myriad of neurological and systemic manifestations are putting us in an unequal position with this fatal disease. Early diagnosis can increase survival and decrease intensity of symptoms. So every new case is important as warning that we must not forget about this disease.


1995 ◽  
Vol 94 (1) ◽  
pp. 48-51 ◽  
Author(s):  
Giovanni Mantovani ◽  
Silvia Esu ◽  
Giorgio Astara ◽  
Biancarosa Lampis ◽  
Antonio Macciò ◽  
...  

Blood ◽  
2008 ◽  
Vol 111 (3) ◽  
pp. 1560-1566 ◽  
Author(s):  
Marie-Cécile Le Deley ◽  
Alfred Reiter ◽  
Denise Williams ◽  
Georges Delsol ◽  
Ilske Oschlies ◽  
...  

Abstract To study prognostic factors of progression/relapse, data concerning 225 children enrolled between 1987 and 1997 in Berlin-Frankfurt-Münster, Société Française d'Oncologie Pédiatrique and United Kingdom Children's Cancer Study Group prospective studies for the treatment of anaplastic large cell lymphoma (ALCL) were merged. Median follow-up was 9.3 years. Five-year overall survival and event-free survival of the whole population was 81% (95% confidence interval, 76%-86%) and 69% (63%-74%), respectively. B symptoms, mediastinal involvement, skin lesions, visceral involvement, St Jude stage 3-4, Ann Arbor stage 3-4, and elevated lactate dehydrogenase increased the risk of progression/relapse in the univariate analysis. In the multivariate analysis, 3 factors remained significant: mediastinal involvement (relative risk [RR] = 2.1 [1.2-3.5]), visceral involvement defined as lung, liver, or spleen involvement (RR = 2.1 [1.3-3.6]), and skin lesions (RR = 1.9 [1.1-3.2]). Five-year progression-free survival (PFS) of the 81 patients with none of these risk factors was 89% [82%-96%], contrasting with a 5-year PFS of 61% [53%-69%] in the 144 patients with at least 1 risk factor (RR = 4.4 [2.2-8.9; P < .001). In conclusion, 3 factors associated with an increased risk of failure in childhood ALCL have been defined: mediastinal involvement, visceral involvement, and skin lesions.


2009 ◽  
Vol 205 (4) ◽  
pp. 283-287 ◽  
Author(s):  
Shih-Sung Chuang ◽  
Yen-Chuan Hsieh ◽  
Hongtao Ye ◽  
Wei-Shou Hwang

2019 ◽  
Vol 7 (3) ◽  
pp. e000691
Author(s):  
Martina Croci ◽  
Stefanie Klausmann ◽  
Jean-Michel Hatt ◽  
Sarah Schmitt ◽  
Monika Hilbe

Because of sudden death of several frogs following the renewal of a terrarium, two giant ditch frogs (Leptodactylus fallax) were submitted for post-mortem examination. The animals displayed extensive erythematous to ulcerative skin lesions as well as multiple granulomas in the liver, kidney and skeletal musculature. In skin lesions as well as in the brownish granulomas, pigmented fungal sclerotic bodies were found in addition to pigmented hyphal structures, though less common. The fungal pathogen, although not further classified in this study was identified as the etiological agent for chromoblastomycosis (synonym: chromomycosis), a leading cause of fatal systemic disease in poikilothermic animals. It is also a cause of chronic skin disease in human beings and therefore a potential zoonotic agent.


Animals ◽  
2019 ◽  
Vol 9 (11) ◽  
pp. 959
Author(s):  
Melody Knock ◽  
Grace A. Carroll

There is increasing interest in utilizing meat inspection data to help inform farmers of the health and welfare of their herds. The aim of this study was to determine whether ante-mortem measures of welfare in beef and dairy cattle (N = 305) were associated with post-mortem measures at a United Kingdom (UK) abattoir. Multiple regression analysis was used to determine the ability of ante-mortem measures of lameness, cleanliness, skin lesions, hair loss and body condition in predicting hot carcass weight and the frequency of carcass bruising. For beef cattle, lameness score (p = 0.04), cleanliness score (p = 0.02) and age (p < 0.001), were predictors of carcass bruise score while lameness score (p = 0.03), body condition (p = 0.01) and sex (p < 0.001) were predictors of hot carcass weight. For dairy cattle, sex (p < 0.001) and slaughter day (p < 0.001) were predictors of carcass bruise score while skin lesion score (p = 0.01), body condition (p < 0.001), age (p < 0.001), slaughter day (p < 0.001) and number of moves (p = 0.01) were predictors of hot carcass weight. These results suggest that recording carcass weight and carcass bruising at meat inspection may have potential as a general indicator of health and welfare status in cattle. However, animal characteristics and variables, such as slaughter day and abattoir staffing, should be taken into account when interpreting the results.


1991 ◽  
Vol 62 (1) ◽  
pp. 26-29 ◽  
Author(s):  
F. W. Huchzermeyer ◽  
K. D.A. Huchzermeyer ◽  
J. F. Putterill

A field outbreak of pox virus infection in juvenile Nile crocodiles (Crocodylus niloticus), in which high morbidity and negligible mortality occurred, is described. Histopathological examination of the skin lesions revealed numerous large intracytoplasmic inclusions in the dermis and a very mild dermal inflammatory reaction. Scanning electron microscopical examination of the skin revealed the presence of large numbers of virus particles in the inclusions. Skin lesions persisted for 5 to 6 months.


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