Pemphigus Vulgaris Localized to the Scalp with Complete Response to Topical Steroids.

Pemphigus vulgaris (PV) is an anti-cadherin autoimmune disease, characterized by the production of anti-desmoglein 3 (and to a lesser extent anti-desmoglein 1) antibodies, producing acanthosis, typically suprabasal, which clinically translates into flaccid blisters and erosions, generally starting on mucous membranes, with subsequent appearance of skin lesions. Only about 25 cases of PV of exclusively cutaneous location have been described in the literature. Treatment with potent topical steroids is usually sufficient to control the symptoms.

Real-World Characteristics and Outcome of Patients Treated With Single-Agent Ibrutinib for Chronic Lymphocytic Leukemia in Spain (IBRORS-LLC Study).

Background: Ibrutinib demonstrated remarkable efficacy and favorable tolerability in patients with untreated or relapsed/refractory (R/R) chronic lymphocytic leukemia (CLL), including those with high-risk genetic alterations. The IBRORS-CLL study assessed the characteristics, clinical management and outcome of CLL patients receiving ibrutinib in routine clinical practice in Spain.

Patients: Observational, retrospective, multicenter study in CLL patients who started single-agent ibrutinib as first-line treatment or at first or second relapse between January 2016 and January 2019.

Bone marrow infiltration by flow cytometry at diffuse large B-cell lymphoma NOS diagnosis implies worse prognosis without considering bone marrow histology.

Background: The significance of discrepant findings between histology (BMB) and flow cytometry (FC) in bone marrow (BM) examination at diffuse large B-cell lymphoma (DLBCL) diagnosis is uncertain.

Methods: We performed a 5-year retrospective single-center study of patients diagnosed by DLBCL not otherwise specified (n = 82), divided into three groups according to BM infiltration at diagnosis: BMB-/FC- (75.6%), BMB+/FC+ (13.

Central Centrifugal Cicatricial Alopecia Following a Patchy Pattern: A New Form of Clinical Presentation and a Challenging Diagnosis for the Dermatologist.

Central centrifugal cicatricial alopecia (CCCA) is included among the primary lymphocytic cicatricial alopecias. The current nomenclature of CCCA suggested by the North American Hair Research Society refers to the traditional clinical presentation pattern of this type of alopecia, which begins in the central area of the scalp and has a progressive and symmetric centrifuge evolution. However, some exceptions should be highlighted, and a new clinical variety of CCCA presenting with patches of hair loss affecting the lateral and posterior scalp has been recently described.

[New mutation in a young woman diagnosed with Niemann-Pick disease type C].

Background And Objetive: To describe a new molecular variant of Niemann-Pick disease type C (NPC) in a 27 year-old patient with splenomegaly and abolition of osteotendinous reflexes.

Material And Methods: NPC1 is the main gene with described mutation in NPC disease. Here we report a case with a new mutation, p.

Eculizumab Treatment in a Patient with Hematopoietic Stem Cell Transplantation-Associated Thrombotic Microangiopathy and Steroid-Refractory Acute Graft Versus Host Disease.

A 30-year-old man with acquired aplastic anemia underwent an HLA-identical bone marrow transplant. He developed a grade III acute graft versus host disease (GVHD) refractory to various lines of treatment. On post-transplant day 196, he was diagnosed with stem cell transplantation-associated thrombotic micro-angiopathy (HSCT-TMA) and he received treatment with eculizumab 900 mg iv weekly for 4 doses followed by a single dose of 1200 mg 2 weeks later.

Von Willebrand Factor Deficiency Corrected by Lung Transplantation.

Background: In experimental models with von Willebrand disease pigs, plasma von Willebrand factor (vWF) was significantly increased after lung transplantation because lung endothelial cells strongly express vWF. However, these findings have not been confirmed in human beings.

Methods: A 26-year-old man with mild vWF deficiency (FvW:antigen 39 IU/dL; FvW:ristocetin cofactor activity 44 IU/dL; factor VIII 99%; normal multimeric plasma vWF pattern) was referred to our institution and underwent bilateral lung transplantation for cystic fibrosis.