From Skin to Blood: Ulcerative Pyoderma Gangrenosum Unveiling Acute Myeloid Leukemia.

While Pyoderma gangrenosum (PG) is commonly associated with hematological disorders such as acute myeloid leukemia (AML), it typically presents concurrently with the hemopathy, mostly in its bullous form, among middle-aged individuals. Here, we report the unusual case of a young female patient who presented with PG in its ulcerative form, three weeks before the onset of AML. A 31-year-old female presented with a one-week history of painful perianal papulopustule that evolved into an irregular ulceration with violaceous borders, mucopurulent serosity, and erythematous surrounding skin. Laboratory work-up demonstrated elevated inflammatory markers and hyperleukocytosis, with no cytopenia, and normal peripheral blood smear. Two weeks later, the ulcer growth was noted with a similar ulceration at a venipuncture site. A complete blood count revealed pancytopenia, with 45% blasts on the peripheral blood smear. Skin biopsies showed an aseptic neutrophilic infiltrate in favor of PG. Intravenous methylprednisolone was administered with rapid resolution of the lesions. However, the patient died shortly after. The post-mortem results of bone marrow aspirate revealed AML, with immunohistochemistry of the skin lesions confirming the clonality of neutrophils derived from the leukemic clone.  This case highlights a distinctive clinical presentation, illustrating the manifestation of PG three weeks before the onset of AML in its ulcerative rather than bullous form, in a young female patient.

Sphenoid plasmacytoma as initial presentation of multiple myeloma-case report.

Plasmacytoma is a rare plasma cell neoplasm. Whether solitary or associated with multiple myeloma (MM), it rarely involves the skull base, particularly the sphenoid bone. We present a unique case of sphenoid bone plasmacytoma secondary to MM, highlighting diagnostic and therapeutic challenges. A 56-year-old female presented with headaches, vomiting, epistaxis, and cranial nerve deficits. Cerebral imaging revealed a 65-mm tumor infiltrating the sphenoid bone and adjacent structures. Subtotal resection was performed using an endoscopic nasal approach. Histopathology revealed plasmacytoma, and diagnostic workup confirmed MM. By the end of biological exploration, relapse of the sphenoid plasmacytoma was observed, and the patient was successfully treated with radiotherapy, immunochemotherapy, and autologous stem cell transplantation. After 18-month follow-up, sustained complete remission was confirmed. Although rare, the diagnosis of plasmacytoma should be considered in cases of skull base tumors. This localization is highly predictive of MM, warranting comprehensive investigations to initiate prompt and adequate management.

Vascular complications of Behçet disease.

Behçet disease is a multi-systemic complex vasculitis with unknown etiology characterized by different clinical involvements, including mucocutaneous, ocular, vascular, articular, neurological, and gastrointestinal manifestations. Growing evidence supports that different phenotypes, characterized by clusters of co-existing involvements, can be distinguished. Namely, the vascular phenotype identifies a specific group of patients who suffer from recurrent inflammatory thrombosis and arterial involvement. Vascular disease develops in up to 40% with a definite male preponderance and is usually an early manifestation. Venous involvement is significantly more common than arterial disease, and lower extremity deep vein thrombosis is its most frequent manifestation. Arterial disease involves mostly pulmonary arteries and aorta and manifests mainly in the form of aneurysms. Glucocorticoids and immunosuppressants are the recommended first-line treatments in vasculo-Behçet. Furthermore, controlled trials are still needed to assess the role of adding anticoagulation to the treatment regimen, with an accent on new oral anticoagulants. Treatment with anti-TNF alpha agents seems promising, but the management strategies are not clear yet.

COVID-19 Associated Coagulopathy and Thrombotic Complications.

The SARS-CoV-2 virus caused a global pandemic within weeks, causing hundreds of thousands of people infected. Many patients with severe COVID-19 present with coagulation abnormalities, including increase D-dimers and fibrinogen. This coagulopathy is associated with an increased risk of death. Furthermore, a substantial proportion of patients with severe COVID-19 develop sometimes unrecognized, venous, and arterial thromboembolic complications. A better understanding of COVID-19 pathophysiology, in particular hemostatic disorders, will help to choose appropriate treatment strategies. A rigorous thrombotic risk assessment and the implementation of a suitable anticoagulation strategy are required. We review here the characteristics of COVID-19 coagulation laboratory findings in affected patients, the incidence of thromboembolic events and their specificities, and potential therapeutic interventions.

Proteomics characterization of CENP-B epitope in Moroccan scleroderma patients with anti-centromere autoantibodies.

BACKGROUND: Anti-centromere auto-antibodies (ACA) have been described as a marker in Systemic sclerosis (SSc) disease. CENP-B is the major centromere auto-antigen recognized by SSc patients with positive ACA. Our aim was to characterize the major epitope involved in the anti-CENP-B immune response of Moroccan SSc patients. PATIENTS AND METHOD: For identification of SSc biomarkers, 80 sera from patients with SSc and systemic lupus erythematosus (SLE) were screened by indirect immunofluorescence test (IIF) to assess the presence of ANA reactivity. Immunoblotting analysis was performed for 11 sera with positive ACA using the N-terminal and C-terminal region of CENP-B protein as antigens. RESULTS: 29 out of 30 (96, 66 %) patients with SSc had positive ANA. 11 out of 30 (36, 67 %) patients were ACA positive and 6 of them produced auto-antibodies against Nt-CENPB antigen. Two of these 6 Nt-CENPB positive sera produced also other auto-antibodies associated to primary biliary cirrhosis. None of all sera tested showed reactivity against Ct-CENPB. CONCLUSION: Our data showed, for the first time in Morocco, that the Nt-CENPB contains a major epitope for Moroccan SSc patients. These findings could provide additional information that would contribute to improving the diagnosis and management of these patients.

Immunological and Clinical Characteristics of Systemic Lupus Erythematosus: A Series from Morocco.

Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease with a high female predominance. To date, studies about SLE in Morocco are few. This retrospective study describes the clinical and immunological features in a series of 50 SLE Moroccan patients in University Hospital Center of Rabat, Morocco, between December 2011 and December 2013. All patients were screened for antinuclear antibodies (ANA) and anti-DNA antibodies by indirect immunofluorescence, followed by identification of anti-extractable nuclear antigen antibodies by ELISA. The female to male ratio was 6.1:1. Mean age was 31.72 years. The main clinical manifestations were arthritis (82%), mucocutaneous manifestations (80%), renal manifestations (50%), and hematological features (46%). Of the mucocutaneous features, the highest frequencies were observed in the malar rash (68%) and photosensitivity (60%). Of the hematological features, lymphopenia was most frequently observed in 30% of patients, followed by hemolytic anemia in 16% and leucopenia and thrombocytopenia in 8%. Central nervous system was involved in 10%. ANA were found in 88%, anti-DNA antibodies in 56%, and anti-Sm antibodies in 50%. Anti-SSA, anti-SSB, anti-Sm/RNP, and anti-Scl70 antibodies were detected in 38%, 10%, 48%, and 8%, respectively. Our data show that, in our patients, the main clinical and immunological features of SLE remain comparable to patients from other Arab countries.

[Atherosclerosis in systemic lupus erythematosus]. / Athérosclérose au cours du lupus érythémateux systémique.

CONTEXT: Evidence from epidemiological studies demonstrates that patients with systemic lupus erythematosus (SLE) are at increased risk for the development of cardiovascular disease. Traditional cardiovascular risk factors' play an important role in this phenomenon but do not account for the entire risk in lupus patients. OBJECTIVES: The incidence and prevalence of cardiovascular events and infraclinical atherosclerosis are reviewed. Combinations of traditional risk factors with lupus-specific and treatment-related variables are detailed. RESULTS: Atherosclerosis is more prevalent and occurs prematurely in lupus patients. Relative risk of myocardial infarction is between 5 to 8 times greater that of general population, and may exceed 50 in women between 35 and 44 years old. SLE was also found as an independent risk factor for subclinical atherosclerosis, and more than one third of lupus patient show evidence of carotid plaques of coronary artery calcifications. Lupus patients have more frequent traditional risk factors compared with general population of similar age and sex. Besides the traditional risk factors, SLE specific risk factors have been identified among witch advanced age at diagnosis, current disease activity, duration of the disease and renal activity. Moreover, lipid abnormalities in patients with SLE are common and likely are one of the major causes of premature atherosclerosis in these patients; the dyslipoprotein associated increased triglycerides and depressed HDL-cholesterol with proinflammatory HDL production. Autoimmunity may have a part of responsibility, but data's in favour of this hypothesis are not strong. Other mechanisms such as vascular inflammation, oxidative stress, immune complexes and complement activation may also elicit endothelial damage and promote atherosclerosis are associated with the pathogenesis of both SLE and atherosclerosis. Steroids may have a true double-edged role with a pro-atherogenic risk regarding the exacerbation of metabolic risk factors and a "beneficial" anti-inflammatory role. It is becoming increasingly apparent that antimalarials treatment in SLE has an atheroprotective and a cardioprotective effect. The other immunosuppressive drugs may reduce progression of atherosclerosis and cardiovascular events but their precise role remains to be elucidated. Despite their role in primary prevention in target general population, for now, systematic prescription of statins does not show a great benefit in the cardiovascular risk in lupus patients. CONCLUSION: Mechanisms of atherosclerosis in SLE remain elusive. It is partially explained by the interaction of traditional cardiovascular risk factors, lupus-specific factors and therapy specially corticosteroids. Management strategies of lupus should include early all those items.

Systemic lupus erythematosus associated with sickle-cell disease: a case report and literature review.

INTRODUCTION: The occurrence of systemic lupus erythematosus has been only rarely reported in patients with sickle-cell disease. CASE PRESENTATION: We describe the case of a 23-year-old North-African woman with sickle-cell disease and systemic lupus erythematosus, and discuss the pointers to the diagnosis of this combination of conditions and also present a review of literature. The diagnosis of systemic lupus erythematosus was delayed because our patient's symptoms were initially attributed to sickle-cell disease. CONCLUSIONS: Physicians should be alerted to the possible association of sickle-cell disease and systemic lupus erythematosus so as not to delay correct diagnosis and initiation of appropriate treatment.

Churg-Strauss syndrome associated with AA amyloidosis: a case report.

Churg Strauss syndrome is a rare systemic and pulmonary vasculitis exceptionally associated with AA amyloidosis. We report the case of a 65-year old woman with past medical history of asthma. She developed polyarthralgia, headache and purpura. A laboratory workout found hypereosinophilia (1150/µL), positive p-ANCA, microscopic haematuria and proteinuria at 2g/day. A diagnosis of Churg-Strauss syndrome was established based on five criteria of the American College of Rheumatology (ACR). Renal biopsy showed an important type AA amyloid deposit. The patient was treated with steroids with a good response of the vasculitis and amyloidosis with disappearance of the proteinuria.

[Aging and geriatric oncology]. / Vieillissement et oncogériatrie.

Chronologic age is not the exclusive factor for therapeutic decision, specially because life expectancy in very old age remains significant. Evaluation of health, autonomy and diseases are more important. Health status in very old age results from genetic factors, life-course and aging. The later may be understood as an adaptative process in response to life-course. These factors lead to frailty, whose evaluation is essential to high quality care in elderly patients. This evaluation must be multidimensional and lead to a care-plan. Even no randomized controlled trial had demonstrated benefits of geriatric intervention in cancer elderly patients, there is a high probability that improvement in mortality, morbidity, autonomy and quality of care may be obtained by strong collaboration of oncologists and geriatricians.