scholarly journals CS-7 A case of Lymphomatoid granulomatosis with skin, lung, and intracranial lesions due to multicentric development

2021 ◽  
Vol 3 (Supplement_6) ◽  
pp. vi28-vi28
Author(s):  
Satoru Komaki ◽  
Takuya Furuta ◽  
Tetsuya Negoto ◽  
Mayuko Moritsubo ◽  
Hideo Nakamura ◽  
...  

Abstract Introduction: LYG is very rare tumor and composed of large EB-positive B cells and reactive T cells. In this study, we experienced a case of LYG with multiple intracranial, cutaneous, and pulmonary masses. We report the pathogenesis and pathophysiology of LYG, including a discussion of the literature. case: A 69-year-old female presented with a growing lump in her lower back that had been present for several years. Six months later, she was found to have multiple masses in her lungs and intracranial region and underwent surgical removal for diagnostic purposes. Intraoperative findings: The tumor was substantial, reddish to grayish-white in color, and the margins of the tumor were whitish and hard, with some areas that could not be detached. Pathological findings: There were no atypical lymphocytes, and a small number of EBER-positive cells were observed. IgVH PCR: IgVH PCR was performed on the skin lesions and intracranial lesions, and bands of different sizes were detected, suggesting that the IgVH clone was present in the polyclonal region. Finally, we diagnosed LYG grade 1. discussion: EB-associated lymphoproliferative disease can lead to polyclonal reactive growth or monoclonal neoplastic growth depending on the balance between morphology and host immunity. The results of IgVH PCR suggest that the skin lesions did not cause multiple metastases, but rather that the enlargement of the skin lesions triggered intracranial and pulmonary lesions in an allo-centric manner. The results of IgVH PCR suggested that the skin lesions did not cause multiple metastases, but rather that the skin lesions grew to cause intracranial and pulmonary involvement in an other-centric manner.

2021 ◽  
Author(s):  
Patricia Volkow ◽  
Leslie Chavez-Galan ◽  
Lucero Ramon-Luing ◽  
Judith Cruz-Velazquez ◽  
Patricia Cornejo-Juarez ◽  
...  

High HHV-8 viral load (VL) in Kaposi Sarcoma (KS) has been associated with severe Immune reconstitution inflammatory syndrome (S-IRIS-KS), which can occur after initiating cART, and is linked with high mortality particularly in patients with pulmonary involvement. We investigate if valganciclovir initiated before cART decreases HHV-8 VL and assess if it reduces the incidence of S-IRIS-KS and its attributable mortality. Methods: Open-label parallel-group randomized clinical trial in AIDS cART naive patients with disseminated KS (DKS) as defined by at least two of the following: pulmonary, lymph-node or gastrointestinal involvement, lymphedema, or equal or more 30 skin lesions. In the experimental group (EG), patients were randomized to valganciclovir 900 mg BID four weeks before cART and continued until week-48; in the control group (CG), cART was initiated on week-0. Non-severe-IRIS-KS was defined as: increase in the number of lesions plus equal or more than one log10 HIV-VL decrease or equal or more than 50 cells/mm3 increase or equal or more than 2-fold rise in baseline CD4+ cells. S-IRIS-KS was defined as abrupt clinical worsening of KS lesions and/or fever after ruling out another infection following cART initiation, and at least three of the following: thrombocytopenia, anemia, hyponatremia, or hypoalbuminemia. Results: 40 patients were randomized and 37 completed the study. In the ITT analysis, the overall mortality did not differ between groups. In the per-protocol analyses, the difference showed a trend for higher S-IRIS-KS mortality in the CG 3/19 (15.7%), compared to EG 0/18 (p=0.07). The incidence of S-IRIS KS was significantly lower in the EG; two patients, one each had S-IRIS-KS episode (0.038 per 100 patient-days) compared to CG group, four patients developed 12 S-IRIS-KS episodes (0.21 per 100 patient-days); incidence rate of 0.09 (95% CI 0.02-0.5 p=0.006). Mortality in patients with pulmonary KS was significantly lower in EG, 3/4 in CG vs 0/5 in EG. S-IRIS-KS was associated with higher HHV-8-VL; IL6 and CRP; valganciclovir was protective. Of survivors at week 48, 82% achieved more than 80% remission. No difference was found between groups in the number of non-S-IRIS-KS events. Conclusions: Valganciclovir significantly reduced the episodes of S-IRIS-KS although attributable KS mortality was lower in the EG the difference was not significant (p=0.07). Mortality was significantly lower in EG patients with pulmonary KS.


2021 ◽  
Vol 5 (1) ◽  
pp. 41-44
Author(s):  
Mohd Hazwan Bin Maznon ◽  
Khairul Abdillah Bin Mohamad ◽  
Siti Zulaifah Binti Che Saidi ◽  
Lim Han Sim ◽  
Ahmad Sabri Bin Omar

A last few decade, case of infection with Mycobacterium tuberculosis (TB) is increasing.  TB infection of other than pulmonary is quite challenging to diagnose due to non-specific signs and symptoms (Wellons III et al., 2004). Spine being the commonest site of skeletal TB which account approximately 50% from reported cases (Tuli, 2002). Spinal TB can be associated with pulmonary infection, however in less than 1% of cases, it occurs in the absence of pulmonary involvement (Davidson & Horowitz, 1970; Gorse, Pais, Kusske, & Cesario, 1983; Omari, Robertson, Nelson, & Chiu, 1989; Rezai, Lee, Cooper, Errico, & Koslow, 1995). TB infection involving the lumbosacral junction is uncommon, only 2 to 3% from all cases of spinal TB (Ahmadi, Bajaj, Destian, Segall, & Zee, 1993; Dayras, Lorilloux, Hugonet, & Benichou, 1985; MANSBERG, ROWE, & WALKER, 1991; Pun et al., 1990; Rajasekaran et al., 1998). Isolated sacral TB is rare entity, The patient usually presented with lower back pain with or without neurological involvement (Patankar et al., 2000). Here we presented a case of sacral TB which not associated with neurological impairment.


2020 ◽  
Vol 8 (2) ◽  
Author(s):  
Sachi Matsubayashi ◽  
Manabu Suzuki ◽  
Keita Sakamoto ◽  
Shinyu Izumi ◽  
Masayuki Hojo ◽  
...  

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Rashid AL Umairi ◽  
Danielle Blunt ◽  
Wedad Hana ◽  
Matthew Cheung ◽  
Anastasia Oikonomou

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare nonmalignant lymphohistiocytic proliferative disorder. We report a patient with RDD who presented with multiple skin lesions, pulmonary involvement, and CT manifestations mimicking Langerhans cell histiocytosis, which improved after initiation of corticosteroid treatment.


1974 ◽  
Vol 60 (4) ◽  
pp. 317-323 ◽  
Author(s):  
Renato Musumeci ◽  
Carlo Uslenghi

Abdominal lymphography was performed in 30 patients, 12 males and 18 females, with sarcoidosis. The diagnosis of disease was in every case histological, after mediastinal biopsy in 16 cases and after biopsy of lymph nodes in various sites in 14 cases. Mediastino-pulmonary involvement of varying degree was present in 23 patients. Lymphography revealed involvement of the inguinoretroperitoneal lymph nodes in 18 cases, bilateral in 15 of them. Lymphographic diagnosis of sarcoidosis is fairly arduous because the pattern elicited is very similar to that of lymphomas. The pathological findings were graded into 4 groups. No correlation between lymphographic pattern and duration and extent of the disease was demonstrated. In 5 patients with pathological lymphography lymph node biopsy confirmed the diagnosis. The routine use of lymphography in patients with sarcoidosis is not to be racommended because the demonstration of extensive lymph node involvement does not affect the treatment in any way.


2020 ◽  
Vol 13 (9) ◽  
pp. e235842
Author(s):  
Stephanie d'Incau ◽  
Maria-Isabel Vargas ◽  
Alexandra Calmy ◽  
Jean-Paul Janssens

Most Mycobacterium fortuitum infections described involve direct inoculation through skin lesions. We describe the case of a patient without risk factors who presented with an intracranial mass and a pulmonary infection with M. fortuitum. As M. fortuitum are rarely pathogens, there is little knowledge about the optimal treatment and outcome of such infections: what is the best mode of administration, what is the best therapy duration and is surgery always required are some of the unanswered questions. In our patient, surgical removal of the mass associated with a 1-year antimycobacterial therapy led to a full recovery. Even though M. fortuitum was rapidly identified in sputum, it was initially considered non-pathogenic and the definitive diagnosis required almost 6 weeks of investigations. New molecular techniques will probably lead to more identifications of M. fortuitum in the next few years and a better knowledge of their possible pathogenicity and optimal treatment.


Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 1612-1612
Author(s):  
Melanie G. Cornejo ◽  
Michael G. Kharas ◽  
Benjamin Lee ◽  
Sandra A. Moore ◽  
Gary Gilliland ◽  
...  

Abstract Molecular pathogenesis of human peripheral T-cell malignancies remains poorly understood. We aimed to establish a model for these disorders by using the recently identified JAK3A572V allele. This mutation, which lies in the pseudokinase (JH2) domain of JAK3, transforms Ba/F3 cells to factor-independent growth and causes a striking lymphoproliferative phenotype in a murine bone marrow transplantation assay. Further characterization of JAK3A572V animals revealed that the aberrantly expanded cell population consisted of a mature effector/memory subtype of CD8+T-cells that infiltrated all major lymphoid and several non-lympoid organs and could be transplanted into secondary and tertiary recipients. These JAK3A572V T-cells had increased proliferative capacities and displayed enhanced phosphorylation of common JAK3 targets, such as STAT5 and S6-kinase. Proliferation of primary T-cells transformed by JAK3A572V was effectively inhibited with a small molecule JAK inhibitor that had no effect on the proliferative potential of control cells transduced with a wildtype JAK3 allele. Furthermore, the mutant cells showed increased production of cytotoxic cytokines, such as IFN-γ and TNF-α, compared to wildtype counterparts, which correlated with an increased cellular cytotoxicity towards allogeneic target cells. Of particular interest, JAK3A572V animals presented with skin lesions and histopathologic analysis showed aberrant skin-homing T-cells tagging along the epidermal/dermal junctions. Mice receiving Rag1-deficient donor cells transduced with the JAK3A572V allele also developed a lethal lymphoproliferative disease characterized by the expansion of immature CD3−TCRβ−CD4+/−CD8+ cells, suggesting that the JAK3A572V-dependent lymphoproliferation does not require proper TCR rearrangement. Altogether these results indicate that in this murine model, constitutive activation of JAK3 results in peripheral/cutaneous T-cell lymphoma (PTCL) that closely resembles the human disease. These findings suggest the possibility that the molecular basis of human PTCL could include aberrant JAK3 signaling and might provide a useful platform for deciphering the molecular and cellular mechanisms and requirements for peripheral lymphoid disease development and progression. Furthermore, it provides an opportunity to investigate the therapeutic potential of selective JAK3 inhibitors for this subset of lymphoid disorders, whose treatment remains a challenge.


2013 ◽  
Vol 5 (1) ◽  
pp. 1 ◽  
Author(s):  
Robert M. Lober ◽  
Anand Veeravagu ◽  
Harminder Singh

Brain tuberculoma has previously accounted for up to a third of new intracranial lesions in areas endemic with tuberculosis, but is unexpected in the United States and other Western countries with improved disease control. Here we show the importance of considering this diagnosis in at-risk patients, even with no definitive pulmonary involvement. We describe a young man who presented with partial seizures and underwent craniotomy for resection of a frontoparietal tuberculoma. He subsequently completed six months of antituberculosis therapy and was doing well without neurological sequelae or evidence of recurrence five months after completion of therapy. With resurgence of tuberculosis cases in the United States and other Western countries, intracerebral tuberculoma should remain a diagnostic consideration in at-risk patients with new space occupying lesions. Mass lesions causing neurological sequelae can be safely addressed surgically and followed with antituberculosis therapy.


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