Sylvia Aide Martínez-Cabriales

Low incidence and severity of graft-versus-host disease after outpatient allogeneic peripheral blood stem cell transplantation employing a reduced-intensity conditioning

Background:  The prevalence and features of graft‐versus‐host disease (GVHD) in patients receiving allografts using peripheral blood stem cells (PBSCs) after a reduced‐intensity conditioning (RIC) regimen are not well known. Several features of GVHD in patients at two institutions using RIC were assessed.

Higher doses of CD34+ progenitors are associated with improved overall survival without increasing GVHD in reduced intensity conditioning allogeneic transplant recipients with clinically advanced disease

The influence of CD34+ cell dose on the outcome of allogeneic peripheral blood stem cell (PBSC) transplantation after reduced intensity conditioning (RIC) remains controversial. The impact of the number of CD34+ hematoprogenitors infused on transplant outcome and on the incidence of graft versus host disease (GVHD) was analyzed.

Long-term results of placental blood allografting using reduced-intensity conditioning: multicenter experience in a developing country

Abstract Background: Placental blood (PLB) hematopoietic stem cell transplantation has recently been explored in an increasing number of patients; the best conditioning regimen has not been established. Material and methods: In an eight-year period, 66 consecutive patients, both children and adults (40 males and 26 females), were grafted with allogeneic placental blood cells using a reduced-intensity conditioning regimen: 23 patients were grafted because of a non-malignant condition and 43 patients for a malignant disease. The median age was 7 years (range 5 months to 72 years). Results: Median time to recover >0·5×109/l granulocytes was 19 days, whereas median time to recover >20×109/l platelets was 23 days. Thirty-eight individuals failed to engraft and they either recovered endogenous hematopoiesis or died. Patients have been followed for periods ranging from 0·5 to 66 months, median 9 months. The median overall post-transplant survival (OS) was 22 months and the 36-month OS was 32%; it was significantly better for individuals grafted with 6/6 matched cords (45%). The cumulative incidences of grade II–IV acute graft-versus-host disease (GVHD) and grade III–IV acute GVHD for the patients who engrafted were 33 and 10%, respectively. Discussion: The low engraftment rate should be improved by selecting better cord blood units; additional studies are needed to define if non-myeloablative conditioning is preferable over conventional conditioning.

Erythema elevatum diutinum: an atypical presentation.

Erythema elevatum diutinum (EED) is classified within the small vessel vasculitis. It is a rare, chronic and progressive disease affecting mostly extensor surfaces and skin overlying the joints.1 Clinically, it presents as multiple erythematous and violaceous papules with a symmetrical and bilateral distribution. Associations with underlying diseases are common, including autoimmune disease, malignancies and infections; although drug-induced cases have been reported. Treatment varies greatly among the literature, with dapsone being the most effective and most frequently used drug.2 EED is an important differential diagnosis of rheumatic disease, especially when treating patients with chronic arthralgias and nonspecific cutaneous lesions, in whom associated diseases must be ruled out.3 We describe a 45-year-old man with chronic joint pain and atypical hyperpigmented macules in palms, as well as euchromatic papules over the ears.

Primary biliary cholangitis associated with warm autoimmune hemolytic anemia.

There are many autoimmune diseases associated with primary biliary cholangitis (PBC), known as primary biliary cirrhosis; however, the association between PBC and warm autoimmune hemolytic anemia (wAIHA) has rarely been reported. It is documented that hemolysis is present in over 50% of the patients with chronic liver disease, regardless of the etiologies. Due to the clear and frequent relationship between PBC and many autoimmune diseases, it is reasonable to suppose that wAIHA may be another autoimmune disorder seen in association with PBC. Here we reported a 53‐year‐old female patient diagnosed with wAIHA associated with PBC.

Cold subcutaneous abscesses as the first manifestation of disseminated coccidioidomycosis in an immunocompromised host.

Coccidioidomycosis is an endemic fungal infection in the southwestern USA and northern Mexico. It is caused by Coccidioides immitis and C. posadasii. This infection occurs due to the inhalation of airborne arthroconidia, causing a mild pulmonary infection, but most cases are asymptomatic. Disseminated coccidioidomycosis (DC) is a rare entity occurring in less than 1% of all cases, usually in immunocompromised patients, and it carries high risks of morbidity and mortality. The skin is one of the most frequently affected organs and in some cases cutaneous lesions may be the first or only sign of infection. A wide spectrum of clinical lesions may develop, including cold abscess. In immunocompromised hosts, DC represents a diagnostic and therapeutic challenge. Treatment is based on antifungal drugs, such as amphotericin B and azoles, administered for long periods of time and under close follow up to monitor the treatment response and to detect relapse. In the following case report, we present a 35‐year‐old male patient with systemic lupus erythematosus under immunosuppressive therapy who presented with cold subcutaneous abscesses as the first sign of DC.

Primary biliary cirrhosis associated with warm‐type autoimmune hemolytic anemia

There are many autoimmune diseases associated with primary biliary cholangitis (PBC), known as primary biliary cirrhosis; however, the association between PBC and warm autoimmune hemolytic anemia (wAIHA) has rarely been reported. It is documented that hemolysis is present in over 50% of the patients with chronic liver disease, regardless of the etiologies. Due to the clear and frequent relationship between PBC and many autoimmune diseases, it is reasonable to suppose that wAIHA may be another autoimmune disorder seen in association with PBC. Here we reported a 53‐year‐old female patient diagnosed with wAIHA associated with PBC.

An Update on Calciphylaxis

Calciphylaxis, also known as calcific uremic arteriolopathy and uremic small artery disease with medial wall calcification and intimal hyperplasia, is a multifactorial cutaneous vascular disease characterized by chronic, painful, non-healing wounds that occur frequently in patients with chronic kidney disease, predominantly in those with end-stage renal disease. The pathogenesis remains unclear, and the development of calciphylaxis lesions depends on medial calcification, intimal fibrosis of arterioles and thrombotic occlusion. Despite an increase in reports of calciphylaxis in the literature and clinical recognition of demographic characteristics and risk factors associated with calciphylaxis, it remains a poorly understood disease with high morbidity and mortality. In this review, we analyze and summarize the clinical manifestations, pathogenesis and pathophysiology, histopathology, differential diagnosis, diagnostic workup and treatment modalities for calciphylaxis. Because of the lack of consensus regarding the optimal approach to and treatment of this disorder, a high degree of clinical suspicion, early diagnosis, and multimodal and multidisciplinary treatment in collaboration with dermatology, nephrology, wound care, nutrition and pain management specialties may improve survival in patients with calciphylaxis.

SkIndia Quiz 38: Subcutaneous thumb tumor

A 58‐year‐old man presented with a 2‐year history of a painless subcutaneous mass on the right thumb [Figure 1a]. The tumor had gradually increased in size and there was no preceding history of trauma. Physical examination revealed a solitary tumor on the thumb tip underneath indurate, pink skin of 1 cm size. A radiograph of the affected part revealed no abnormality. Histological examination revealed a mass composed of spindle‐shaped cells [Figure 1b and c].

A man with abdominal pain and skin pigmentation

A 52-year-old man presented to the Emergency department because of 1-week history of nausea and abdominal pain. The patient referred generalized muscle pain, fatigue, hyporexia and 13 kg weight loss since 18 months ago. He also noticed a brown-colored pigmentation in his skin during the last year. Laboratory data showed an elevated white blood cell count (11,300/μL, 7900 neutrophils), hemoglobin 14.5 mg/dL, potassium 5.37 mmol/L, sodium 135.7 mmol/L, magnesium 2.71 mg/dL, phosphorus 4.7 mg/dL, calcium 9.4 mg/dL, glucose 70 mg/ dL, BUN 21 mg/dL, VIH negative. On a physical examination blood pressure was 90/50 mmHg, hyperpigmentation of the skin mostly in palmar creases,flexural areas, nipples, vermilion border of the lips and the buccal periodontal was noticed (Fig. 1).