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.

Coeliac Disease: A Rare Cause of Myocardial Infarction With Non-Obstructive Coronary Arteries.

Myocardial infarction with non-obstructive coronary arteries (MINOCA) poses a diagnostic dilemma. Identifying the underlying etiology is essential to ensuring appropriate management. Cardiac magnetic resonance (CMR) is a valuable tool that can aid clinicians for that purpose. Coeliac disease (CD) is characterized by hypercoagulability and a thrombotic state and represents an exceptional cause of MINOCA. We report the case of a 28-year-old woman who presented with chest pain. The diagnosis of non-ST-elevation MI was obtained based on ECG abnormalities and elevated troponin levels. Coronary angiography was normal. CMR showed late gadolinium enhancement in the lateral left ventricular wall, confirming the diagnosis of MINOCA. A duodenal biopsy allowed the diagnosis of CD. Anticoagulation and a gluten-free diet proved beneficial, with a good outcome after a five-year follow-up. This case highlights the essential role of CMR in MINOCA investigations and the importance of thorough etiological assessment in young patients with no cardiovascular risk factors.

Bi-lineage B- and T-lymphoid Extramedullary Blast Crisis at an Initial Presentation of Chronic Myeloid Leukemia: A Case Report and Literature Review of Extramedullary Blast Crisis.

Chronic myeloid leukemia (CML) with BCR-ABL1-positive cells is a myeloproliferative neoplasm (MPN) characterized by a chromosomal translocation t(9,22)(q34.1;q11.2), which results in the formation of a Philadelphia (Ph) chromosome containing the BCR-ABL1 fusion gene. Extramedullary blast crisis (EBC) associated with bcr/abl-positive CML is a rare initial presentation. Here, we present and discuss the case of a 51-year-old man who presented with a weight loss history, cervical swelling, and left-sided abdominal pain. He had a white blood cell count of 147,910/mm3. The blood smear study revealed myelemia in 23% and 8% of blast-like cells. The bone marrow aspiration and biopsy showed a richly cellularized sample; the megakaryocytes were present; the granular neutrophil line was at 89% with blasts at 1%. The cytogenetic analysis revealed a complex karyotype with the presence of a Philadelphia chromosome t (9, 22) (q34, q11) associated with additional cytogenetic abnormalities (ACA). Molecular analysis (PCR) detected a BCR::ABL1 (p210) rearrangement. At this point, a diagnosis of CML in the chronic phase was confirmed, but a cervical lymph node biopsy analysis revealed a bi-phenotypic B/T-lymphoblastic lymphoma (LBL) and expressed at fluorescent in situ hybridization (FISH) analysis BCR::ABL1 rearrangement. These findings were consistent with the diagnosis of a bi-phenotypic B/T extramedullary blast crisis associated with CML.

Impact of a psychoeducative intervention on adherence to HAART among low-literacy patients in a resource-limited setting: the case of an Arab country--Morocco.

Research has demonstrated that strict adherence is necessary to maximize highly active antiretroviral therapy (HAART) benefits. This is particularly challenging for low-literacy populations in resource-limited settings like Morocco and motivated the implementation of a psychoeducative program for patients under HAART at Rabat University Hospital. The study aimed at assessing the program's impact on adherence to antiretroviral medication, knowledge of HIV/AIDS and HAART, quality of life, and biological parameters. It included patients under treatment for at least 2 months that benefited from 3 to 5 educational and psychological support sessions. Data were collected at baseline, 3 and 6 months. In all, 50 patients were included. The mean age was 38 years; 52% were illiterate and 62% unemployed. Adherence scores were high at baseline (98%) and showed no significant change throughout the study. Knowledge of HAART and HIV/AIDS, and quality of life improved significantly both at months 3 and 6. Significant increase for CD4 count rates and decrease for viral load rates were also reported. The program had no significant impact on adherence but substantively developed patients' knowledge of HIV/AIDS and HAART and improved their quality of life.

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.

Immature platelets: a review of the available evidence.

Immature platelets or reticulated platelets are newly released thrombocytes. They can be identified by their large size and high RNA cytoplasm concentration. Immature platelet fraction (IPF) represents the percentage of immature circulative platelets to the total number of platelets. The development of analytical standardization of this hematological parameter by new automated devices allowed a better exploration of its contribution in a context of thrombocytopenia. In fact, several studies had confirmed its clinical utility to differentiate immune thrombocytopenia from other causes of thrombocytopenia. IPF can also predict platelets recovery after chemotherapy and successful engraftment. In addition, immature platelets have shown utility in other diseases such as coronary artery diseases, bacterial infections and liver diseases. Despite all these advantages, immature platelet fraction can be increased in some cases of thrombocytopenia characterized by platelets hypoproduction. The aim of this review is to present the immature platelet fraction contribution in clinical practice.

Challenges for lupus management in emerging countries.

In emerging countries, systemic lupus erythematosus (SLE) has been associated with several unfavorable outcomes including disease activity, damage accrual, work disability and mortality. Poor socioeconomic status (SES) and lack of access to healthcare, especially in medically underserved communities, may be responsible for many of the observed disparities. Diagnostic delay of SLE or for severe organ damages (renal involvement) have a negative impact on those adverse outcomes in lupus patients who either belong to minority groups or live in emerging countries. Longitudinal and observational prospective studies and registries may help to identify the factors that influence poor SLE outcomes in emerging countries. Infection is an important cause of mortality and morbidity in SLE, particularly in low SES patients and tuberculosis appears to be frequent in SLE patients living in endemic areas (mainly emerging countries). Thus, tuberculosis screening should be systematically performed and prophylaxis discussed for patients from these areas. SLE treatment in the developing world is restricted by the availability and cost of some immunosuppressive drugs. Moreover, poor adherence has been associated to bad outcomes in lupus patients with a higher risk of flares, morbidity, hospitalization, and poor renal prognosis. Low education and the lack of money are identified as the main barrier to improve lupus prognosis. Newer therapeutic agents and new protocols had contributed to improve survival in SLE. The use of corticoid-sparing agents (hydroxychloroquine, methotrexate, azathioprine and mycophenolate mofetif) is one of the most useful strategy; availability of inexpensive generics may help to optimize access to these medications.

[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.

Cardiac thrombosis as a manifestation of Behçet syndrome.

Herein, we report 3 cases of Behçet syndrome that were accompanied by intracardiac thrombus. The 1st patient was a 30-year-old man who presented with dyspnea; a right atrial thrombus was identified upon transthoracic echocardiography. The 2nd patient was a 52-year-old man who was admitted for dyspnea; transthoracic echocardiography revealed an echogenic mass in the right ventricle. The 3rd patient was a 23-year-old man who was hospitalized for hemoptysis; the diagnosis of pulmonary embolism was made, and right ventricular thrombosis was found.Because these patients had no hemodynamic compromise, medical management consisting of immunosuppressive and anticoagulative therapy was adopted. This treatment resulted in complete dissolution of the thrombi.

Recurrent meningitis revealing a Behçet's disease.

The aim of the present study was to describe a case of Behçet's disease revealed by a recurrent meningitis and to review literature on these two conditions. We describe the case of a 25-year-old man who presented four episodes of recurrent meningitis without any locoregional cause and developed oral and genital ulcerations few months later. Behçet's disease is a chronic, multisystemic disorder with variable prevalence in different geographical areas. Its neurological manifestations are well recognized. Both central and peripheral nervous systems can be involved. Recurrent meningitis in Behçet's disease is exceptional. To our knowledge, only two cases reported recurrent meningitis as initial manifestation of Behçet's disease. This case report underscores another facet of neurological manifestations of Behçet's disease.