scholarly journals Case Report: Intestinal Nodular Lymphoid Hyperplasia as First Manifestation of Activated PI3Kδ Syndrome Due to a Novel PIK3CD Variant

2021 ◽  
Vol 9 ◽  
Author(s):  
Antonio Marzollo ◽  
Silvia Bresolin ◽  
Davide Colavito ◽  
Alice Cani ◽  
Paola Gaio ◽  
...  
Keyword(s):  
Case Report ◽  
Complete Remission ◽  
Clonal Expansion ◽  
Lymphoid Hyperplasia ◽  
Lymphoproliferative Disease ◽  
Lymphoid Cells ◽  
The Novel ◽  
First Line ◽  
Nodular Lymphoid Hyperplasia ◽  
Gut Mucosa

Nodular lymphoid hyperplasia (NLH) is a lymphoproliferative disease caused by non-clonal expansion of lymphoid cells in the gut mucosa. Little is known about the pathogenesis of NLH, which is often disregarded as an insignificant or para-physiologic phenomenon. We present the case of a girl with isolated diffuse NLH (extending from the stomach to the rectum) caused by activated PI3Kδ syndrome (APDS) due to the novel p.Glu525Gly variant in PIK3CD. The gain-of-function effect of the variant was confirmed by demonstration of over activation of the Akt/mTOR pathway in the patient's cells. APDS diagnosis led to treatment with sirolimus, which resulted in the complete remission of NLH and in the prevention of extra intestinal complications. In conclusion, we identify APDS as a novel cause of isolated NLH and suggest that patients with severe pan-enteric NLH should be screened for this disorder that may not be apparent on first-line immunological testing.

Nodular lymphoid hyperplasia: rare case of lymphoproliferative disease in the lung

2009 ◽  
Vol 57 (6) ◽  
pp. 324-327 ◽  
Author(s):  
Yoko Karube ◽  
Masayuki Chida ◽  
Koichi Honma ◽  
Osamu Araki ◽  
Satoru Kobayashi ◽  
...  
Keyword(s):  
Rare Case ◽  
Lymphoid Hyperplasia ◽  
Lymphoproliferative Disease ◽  
Nodular Lymphoid Hyperplasia

P.05.12 DIFFUSE INTESTINAL NODULAR LYMPHOID HYPERPLASIA (DNLH) IN A PATIENT WITH EPIGASTRIC PAIN: A CASE REPORT

2016 ◽  
Vol 48 ◽  
pp. e152
Author(s):  
E. Capoferro ◽  
E. Ntakirutimana ◽  
D. Dal Fior ◽  
R. Colombari ◽  
P. Inturri ◽  
...  
Keyword(s):  
Case Report ◽  
Epigastric Pain ◽  
Lymphoid Hyperplasia ◽  
Nodular Lymphoid Hyperplasia

Classical Hodgkin lymphoma-like post-transplant lymphoproliferative disease after allogeneic stem cell transplantation for primary myelofibrosis is successfully treated with nivolumab: A case report

2020 ◽  
pp. 107815522094646
Author(s):  
Ahmet K. Gunes ◽  
Ilknur Demir ◽  
Mustafa Pehlivan
Keyword(s):  
Stem Cell ◽  
Case Report ◽  
Complete Remission ◽  
Stem Cell Transplantation ◽  
Hodgkin Lymphoma ◽  
Primary Myelofibrosis ◽  
Lymphoproliferative Disease ◽  
Brentuximab Vedotin ◽  
Classical Hodgkin Lymphoma ◽  
Post Transplant

Introduction Post-transplant lymphoproliferative disease (PTLD), a lymphoid proliferation observed after the solid organ transplantation or allogeneic stem cell transplant, is an important and mortal complication that can occur during the post-transplant period. Classical Hodgkin lymphoma-like PTLD is the least form of PTLD. We are presenting an adult case of classical Hodgkin lymphoma-like PTLD which was successfully treated with nivolumab. Case report A 31-year-old female was diagnosed with primary myelofibrosis and we performed allogeneic stem cell transplantation from her HLA fully matched brother in 2015. Two years after transplant, classical Hodgkin lymphoma-like PTLD was diagnosed. The patient was resistant to six cycles of ABVD chemotherapy and four cycles of brentuximab vedotin. Management and outcome After the failure of ABVD and brentuximab vedotin, we started nivolumab therapy at a dose of 3 mg/kg every 2 weeks. After six cycles, we achieved a PET negative complete remission. After 10 cycles of nivolumab, the patient is still followed with a complete remission. Still, there is no evidence of acute or chronic GvHD, and therefore no need for immunosuppressive treatment. No auto-immune complication was observed. It is planned to give nivolumab treatment to the patient until the progression. Discussion Our case has depicted that the classical Hodgkin lymphoma type PTLD may be resistant to the conventional treatments and anti-CD30 brentuximab vedotine. In such cases, nivolumab may be an effective and worth assessing agent in terms of both activity and safety profile.

scholarly journals Treatment of Acute Lymphoid Leukemia Refractory to Classic First-Line and Rescue Protocols

2020 ◽  
Author(s):  
Flávia Tobaldini Russo ◽  
Maria Cristina Martins de Almeida Macedo ◽  
Pedro Amoedo Fernades1 ◽  
Larissa Yukari Okada ◽  
Lucas Augusto Monetta da Silva ◽  
...  
Keyword(s):  
Case Report ◽  
Acute Lymphoblastic Leukemia ◽  
Gold Standard ◽  
Lymphoblastic Leukemia ◽  
Lymphocytic Leukemia ◽  
Lymphoid Cells ◽  
Response To Treatment ◽  
First Line ◽  
Lymphoid Leukemia ◽  
No Gold Standard

Acute Lymphoblastic Leukemia is a very aggressive malignant disorder of lymphoid cells in adults, with recurrence (30 to 60% of the cases) after the initial treatment. Until this moment, there is no gold standard therapy for the treatment of adult patients with acute relapsed/refractory lymphoblastic leukemia. In this case report, we describe two cases of relapsed leukemia: one of lymphocytic leukemia B and one of trilineage leukemia, which presented a satisfactory response to treatment with Bortezomib associated with Vincristine, Dexamethasone, and Bendamustine.

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