Pseudomicroangiopathic Thrombotic Syndrome: Unveiling the Vitamin B12 Deficiency Connection.

Pernicious anemia, a manifestation of vitamin B12 deficiency, can present with a spectrum of hematological abnormalities, sometimes mimicking more severe conditions such as thrombotic microangiopathy (TMA). This case report details a 53-year-old female who presented with significant weight loss, watery diarrhea, and jaundice. Laboratory investigations revealed pancytopenia, hemolysis, and schistocytes, initially suggesting a diagnosis of microangiopathic hemolytic anemia (MAHA). However, significantly low vitamin B12 levels and subsequent bone marrow examination confirmed pernicious anemia with megaloblastic changes. This case underscores the importance of considering vitamin B12 deficiency in the differential diagnosis of patients presenting with TMA-like symptoms. Early recognition and treatment with vitamin B12 supplementation led to rapid clinical improvement and the resolution of symptoms. This report highlights the need for heightened clinical awareness of atypical presentations of pernicious anemia to prevent misdiagnosis and ensure timely, effective treatment.

Bronchial Fissuring: A Severe Complication of Arterial Aneurysm.

Arterial aneurysms are rare and may occur in the context of Behçet's disease. The natural progression of these aneurysms can lead to an increase in their size and eventual rupture into the bronchi, causing life-threatening hemoptysis. We report a case of a subclavian artery aneurysm in a 30-year-old female patient with Behçet's disease who presented with moderate hemoptysis caused by a fistulized left subclavian artery aneurysm into the left main bronchus. The patient was treated with a bolus of corticosteroids followed by oral therapy, and six boluses of cyclophosphamide were scheduled. Unfortunately, the patient's condition deteriorated, and she died after her second cycle of cyclophosphamide following fulminant hemoptysis. The management of aneurysms in Behçet's disease is not standardized, but embolization appears to be the most promising conservative therapy.

Primary Breast Tuberculosis Concealed Behind Granulomatous Mastitis.

Granulomatous mastitis is an inflammatory disease that often affects women with a history of breastfeeding. The pathogenesis is still unclear and several factors have been incriminated, such as trauma, metabolic and hormonal disorders, infections, and autoimmunity. This poses a diagnostic issue, given that there are several different diagnoses, particularly carcinomatous mastitis. We report the case of a 32-year-old woman, with a history of breastfeeding, who presented with inflammatory left breast. The physical examination has objectified a 10/10 cm painless mass and a 3 cm homolateral axillary lymphadenopathy. A sonomammography revealed inflammatory left breast infiltration with multiple collections associated with homolateral axillary lymphadenopathies. A Trucut biopsy was performed, revealing granulomatous mastitis without signs of malignancy. Interferon-gamma measurement and Koch Bacillus (BK) search by polymerase chain reaction (PCR) in the breast collection were all negative. The patient was put on non-specific antibiotics with no response and clinical worsening; therefore, we were obliged to start bacillary treatment. The evolution was marked by a total drought and the disappearance of inflammatory signs within a few weeks. Mammary tuberculosis poses a diagnostic issue given the difficulty to identify the bacteria in the samples. This is why tuberculosis should never be excluded despite negative results, especially in endemic countries.

Prevalence of and Risk Factors for Suicide Attempts Among Patients With Severe Psychiatric Disorders in Eastern Morocco.

Background: Suicide attempts are common in patients with severe psychiatric disorders; however, they are rarely studied in this population. Aims: To investigate the prevalence and risk factors associated with suicide attempts among patients with severe psychiatric disorders. Method: This is a cross-sectional study of patients admitted to the Mohammed VI University Hospital of Psychiatry in Oujda, Morocco. Results: A total of 250 patients with a psychiatric disorder were recruited in this study. Among these, 78 cases (31.2%) had a personal history of suicide attempts. A personal history of suicide attempt was significantly higher among women compared to men (45.5% vs. 27.2%, p = .0099). The most common method of suicide attempts was jumping from heights (31%). Patients with a personal history of suicide attempts had a significantly higher prevalence of alcohol consumption (p = .0063), family history of psychiatric disorders (p = .002), family history of suicide attempt (p = .00004), and family history of suicide (p = .018) compared to those who had never made suicide attempts. Limitations: As suicidal behavior is highly stigmatized in Morocco, the number of patients who have made a suicide attempt may be underestimated. Conclusion: Our findings justify the need to provide specialized support to psychiatric patients with risk factors for suicide attempts.

Focal Segmental Glomerulosclerosis Followed by Granuloma and Preceding T-Cell Lymphoma by 46 Months: A Continuation Process or Coincidence.

Focal segmental glomerulosclerosis is a severe renal disease with a complex and unclear pathophysiology. Nephrotic syndrome is the clinical presentation of this renal disease. The recurrence of the disease after renal transplantation and the remission obtained after immune-adsorption treatment illustrate the implication of a circulating factor that requires characterization. Granulomatous inflammation is a tissue reaction caused by several conditions, including neoplastic diseases. In the literature, focal segmental glomerulosclerosis and granulomatous inflammation have both been associated with lymphoma. We report the case of a 56-year-old woman who initially developed focal and segmental glomerulosclerosis. After one year, the granulomatous inflammation was treated as tuberculosis infection and then as sarcoidosis. Finally, after another year, non-specified peripheral T-cell lymphoma was diagnosed.

Chronic lymphocytic leukemia, a rare cause of spontaneous rupture of the spleen.

INTRODUCTION: Spleen Spontaneous Rupture SRS is a rare phenomenon in which the spleen ruptures without associated trauma. This pathology is rarely caused by Chronic Lymphocytic Leukemia. PRESENTATION OF THE CASE: We present a case of a 59-year-old male patient with chronic Lymphocytic Leukemia, who was admitted with an acute abdomen whose clinical and paraclinical examinations revealed a spleen spontaneous rupture. The treatment consisted of a splenectomy. DISCUSSION: Spontaneous spleen rupture was first described by Rokitansky in 1861 and mentioned in many cases since, the common causes of non-traumatic Splenic rupture include myeloproliferative diseases, vasculitis, and infections. However, Chronic Lymphocytic Leukemia (CLL) remains an obscure cause of splenic rupture that requires unique attention. The diagnosis of splenic rupture should be considered in all patients with hematologic malignancies presenting with abrupt onset of abdominal pain, hemodynamic instability, or acute anemia. The choice between conservative treatment and splenectomy depends on different variables: the etiology of the SRS, the hemodynamic stability, the amount of packed red blood cells transfused. Thus, an interventional approach can be advocated for a spontaneous splenic rupture over nonoperative management. Splenic embolization can provide patients with the advantages of both operative splenectomy and conservative management. The mortality rate from SRS is 12.2 %. Neoplastic pathologies were most significantly associated with fatal outcomes. CONCLUSION: The high mortality rate seems to be mainly related to the delayed diagnosis and/or the severity of the underlying pathology. Given its seriousness, it requires a rapid diagnosis and adapted management.

Chronic Inflammatory Orbitopathy Hiding Orbital Lymphoma.

The incidence of lymphoma is constantly increasing worldwide. The reasons for this increase are unclear and likely multiple. B cell lymphomas represent the majority of non-Hodgkin lymphomas. Primary orbital localization remains a rare entity to think about in order to avoid missing a therapeutic emergency. In this article, we report the case of a 52-year-old man who has been treated for five years for an inflammatory orbitopathy with steroids, but the worsening of the clinical condition and the installation of exophthalmia led to push investigations and the outcome was a primary orbital lymphoma marginal zone type. The patient was treated by systemic chemotherapy with immunotherapy (RCHOP protocol) with a very good evolution and complete disappearance of the lesion after chemotherapy.

Primary Cutaneous Hodgkin's Lymphoma: An Extremely Rare Entity.

Skin involvement in Hodgkin's lymphoma (HL) is rare. The diagnosis can be difficult, mainly due to the wide range of cutaneous lesions that can be observed, but also due to the differential diagnosis, even after the immunohistochemical staining. We present the case of a 30-year-old man who presented with a painful cutaneous nodular lesion; biopsy and immunohistochemical stains were consistent with classic HL. The patient was treated with adriamycin, bleomycin, vinblastin, and dacarbazine (ABVD) with complete remission.

Retroperitoneal Fibrosis: Beware of Lymphoma.

Retroperitoneal fibrosis is a rare disease manifesting as chronic soft tissue fibrosis in the retroperitoneum, with potential anatomic and/or functional compromise of adjacent organs. It can be primary (idiopathic) or secondary to other conditions such as cancers, radiotherapy, surgery, traumatisms, infections, autoimmune disorders, or drugs. We report herein a 54-year-old patient with symptomatic retroperitoneal fibrosis leading to bilateral hydronephrosis and renal failure, in whom, after a complex diagnostic workup and protracted clinical course, a follicular lymphoma in the retroperitoneal was identified. The patient was treated with rituximab and cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy protocol, with a resolution of hydronephrosis and lower back pain. We include a thorough literature review on etiopathogenesis, diagnosis, therapy, and prognosis of retroperitoneal fibrosis. A meticulous search for malignancy is necessary for this rare condition that, if positive, may have significant therapeutic and prognostic implications.

Acute spontaneous subdural hematoma as an inaugural presentation of systemic lupus erythematosus with acquired factor XIII deficiency: a case report.

Acute spontaneous subdural hematoma is a rare clinical situation. Among its various etiologies, underlying coagulopathy is associated with a considerable risk of mortality. A 43-year-old female patient with no comorbidity and no personal or family history of bleeding disorders, consulted for acute and intense headache. The brain computed tomography (CT) scan showed a compressive left fronto-parietal acute subdural hematoma. The cerebral magnetic resonance angiography and routine hemostasis workup were normal. Factor XIII activity was low at 41% and the etiological investigation was consistent with the diagnosis of systemic lupus erythematosus. Surgical evacuation of the hematoma, factor XIII supplementation and systemic corticosteroid therapy with hydroxy chloroquine resulted in a favorable outcome. Acquired factor XIII deficiency should be systematically investigated for any acute spontaneous subdural hematoma with a normal hemostasis assessment in an adult with no personal or family history of hemorrhage.

A Case Report of a Primary Conjunctival Diffuse Large B-Cell Lymphoma: Keeping an Eye out Following a Nasopharyngeal Carcinoma.

Primary diffuse large B cell lymphoma of the conjunctiva is a rare disease. In this article, we report the case of a 40-year-old man who had previously been treated with chemotherapy and radiotherapy for undifferentiated carcinoma of nasopharyngeal type (UCNT) and who subsequently developed conjunctival lymphoma. We underline through this observation the importance of thinking about a secondary cancer post-radio-chemotherapy even when the clinical presentation is atypical.

Raynaud's Phenomenon: Beware of Cancers!

Raynaud's phenomenon (RP) is a frequent syndrome and often indicative of connectivitis or hemopathy. The association with solid cancers is exceptional. We report the observation of a patient hospitalized for severe RP whose etiological assessment revealed the existence of colorectal cancer. We discuss, through this clinical case, the potential physiopathological links and underline the importance of looking for underlying cancer in the face of severe, refractory to treatment, or atypical RP.

Acute Myeloid Leukemia With Systemic Lupus Erythematosus: Rare Association or Coincidence.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder due to autoantibodies directed against nuclear and cytoplasmic antigens that may affect several different organs. The association of SLE and acute myeloid leukemia (AML) is rare, the incidence of this combination is not known, but there are few case reports in the literature. We report here the case of a 62-year-old woman, admitted for aetiological diagnosis of weight loss and severe anemic syndrome. The blood count has objectified a pancytopenia with lymphopenia. A thorough assessment was carried out following which a diagnosis of AML associated to SLE was retained. The patient received a low dose of cytarabine due to comorbidities and poor performance status associated with steroids and she died three months after diagnosis with a septic shock.

Morocco's First Biobank: Establishment, Ethical Issues, Biomedical Research Opportunities, and Challenges.

BACKGROUND: Biobanks are highly organized infrastructures that allow the storage of human biological specimens associated with donors' personal and clinical data. These infrastructures play a key role in the development of translational medical research. In this context, we launched, in November 2015, the first biobank in Morocco (BRO Biobank) in order to promote biomedical research and provide opportunities to include Moroccan and North African ethnic groups in international biomedical studies. Here, we present the setup and the sample characteristics of BRO Biobank. METHODS: Patients were recruited at several departments of two major health-care centers in the city of Oujda. Healthy donors were enrolled during blood donation campaigns all over Eastern Morocco. From each participant, personal, clinical, and biomedical data were collected, and several biospecimens were stored. Standard operating procedures have been established in accordance with international guidelines on human biobanks. RESULTS: Between November 2015 and July 2020, 2446 participants were recruited into the BRO Biobank, of whom 2013 were healthy donors, and 433 were patients. For healthy donors, the median age was 35 years with a range between 18 and 65 years and the consanguinity rate was 28.96%. For patients, the median age was 11 years with a range between 1 day and 83 years. Among these patients, 55% had rare diseases (hemoglobinopathies, intellectual disabilities, disorders of sex differentiation, myopathies, etc.), 13% had lung cancer, 4% suffered from hematological neoplasms, 3% were from the kidney transplantation project, and 25% had unknown diagnoses. The BRO Biobank has collected 5092 biospecimens, including blood, white blood cells, plasma, serum, urine, frozen tissue, FFPE tissue, and nucleic acids. A sample quality control has been implemented and suggested that samples of the BRO Biobank are of high quality and therefore suitable for high-throughput nucleic acid analysis. CONCLUSIONS: The BRO Biobank is the largest sample collection in Morocco, and it is ready to provide samples to national and international research projects. Therefore, the BRO Biobank is a valuable resource for advancing translational medical research.

Effective Voriconazole in an Immunocompetent Patient With Amphotericin B Resistant Systemic Cryptococcal Granulomatosis.

The diagnostic management of systemic granulomatosis is a difficult clinical exercise. The most frequent etiologies are tuberculosis and sarcoidosis. However, it is important to search as well for the other causes of granulomas, especially infections and malignancies, the prognosis of which can be poor without adequate treatment. A 67-year-old immunocompetent patient presented with granulomatous adenitis without caseous necrosis. The etiological evaluation had revealed neurological, pulmonary and lymph node systemic cryptococcosis. Conventional antifungal therapy with the triple combination Amphotericin B - Flucytosine - Fluconazole has not been effective, indicating administration of voriconazole. The evolution was rapidly favorable with apyrexia after 48 hours, disappearance of clinical symptoms, normalization of biological parameters of cerebrospinal fluid (CSF) and major improvement of radiological abnormalities. This clinical case is original by the disseminated involvement, the patient's non-immunocompromised status and the primary resistance to amphotericin B. Our findings underline the importance of carrying out an exhaustive evaluation, reflecting on cryptococcosis in any systemic granulomatosis and knowing the various therapeutic alternatives, in particular, voriconazole if primary response to amphotericin B has not been obtained.

Practical Approach for the Management and Evaluation of Paraneoplastic Syndromes.

Paraneoplastic syndromes (PNS) are conditions linked to the presence of tumors, most often malignant, without being the direct translation of a locoregional extension or distant metastases. They affect 10% to 15% of cancer patients, can appear before, after, or simultaneously with a cancer diagnosis, and primarily affect the nervous system, endocrine glands, and skin. The main tumors that provide PNS are lung cancer, gynecological tumors, and lymphomas. The diagnostic and therapeutic approaches are very heterogeneous due to the physiopathological specificities of each type of PNS. The main advances made in recent years have focused mainly on diagnostic tools, which have become more efficient in the diagnosis of PNS and underlying cancers.

Concomitant primary hyperparathyroidism and systemic lupus erythematosus: coincidence or not? A new case report.

Primary hyperparathyroidism (PHP) is the most common cause of hypercalcemia. Patients with systemic lupus erythematosus (SLE) can develop hypercalcemia but it is exceptionally due to PHP. There are only few cases of concurrent SLE and primary hyperparathyroidism (PHP) described in the literature. We report a case of a 31-year-old patient having SLE with lupus nephritis class III and anti-phospholipid syndrome, complicated by pulmonary embolism associated to primary hyperparathyroidism causing severe hypercalcemia and osteoporosis. Even if there is no evidence for potential pathogenic association between PHP and SLE, the recognition of this association is very important because of therapeutic and prognostic impact. Early detection of PHP leads to avoid severe complications and significant morbidity.

State of Art of Idiosyncratic Drug-Induced Neutropenia or Agranulocytosis, with a Focus on Biotherapies.

INTRODUCTION: Idiosyncratic drug-induced neutropenia and agranulocytosis is seldom discussed in the literature, especially for new drugs such as biotherapies outside the context of oncology. In the present paper, we report and discuss the clinical data and management of this relatively rare disorder, with a focus on biotherapies used in autoimmune and auto-inflammatory diseases. MATERIALS AND METHODS: A review of the literature was carried out using the PubMed database of the US National Library of Medicine. We searched for articles published between January 2010 and May 2019 using the following key words or associations: "drug-induced neutropenia", "drug-induced agranulocytosis", and "idiosyncratic agranulocytosis". We included specific searches on several biotherapies used outside the context of oncology, including: tumor necrosis factor (TNF)-alpha inhibitors, anti-CD20 agents, anti-C52 agents, interleukin (IL) 6 inhibitors, IL 1 inhibitors, and B-cell activating factor inhibitor. RESULTS: Idiosyncratic neutropenia remains a potentially serious adverse event due to the frequency of severe sepsis with severe deep tissue infections (e.g., pneumonia), septicemia, and septic shock in approximately two-thirds of all hospitalized patients with grade 3 or 4 neutropenia (neutrophil count (NC) ≤ 0.5 × 109/L and ≤ 0.1 × 109/L, respectively). Over the last 20 years, several drugs have been strongly associated with the occurrence of idiosyncratic neutropenia, including antithyroid drugs, ticlopidine, clozapine, sulfasalazine, antibiotics such as trimethoprim-sulfamethoxazole, and deferiprone. Transient grade 1-2 neutropenia (absolute blood NC between 1.5 and 0.5 × 109/L) related to biotherapy is relatively common with these drugs. An approximate 10% prevalence of such neutropenia has been reported with several of these biotherapies (e.g., TNF-alpha inhibitors, IL6 inhibitors, and anti-CD52 agents). Grade 3-4 neutropenia or agranulocytosis and clinical manifestations related to sepsis are less common, with only a few case reports to date for most biotherapies. Special mention should be made of late onset and potentially severe neutropenia, especially following anti-CD52 agent therapy. During drug therapy, several prognostic factors have been identified that may be helpful when identifying 'susceptible' patients. Older age (>65 years), septicemia or shock, renal failure, and a neutrophil count ≤0.1 × 109/L have been identified as poor prognostic factors. Idiosyncratic neutropenia should be managed depending on clinical severity, with permanent/transient discontinuation or a lower dose of the drug, switching from one drug to another of the same or another class, broad-spectrum antibiotics in cases of sepsis, and hematopoietic growth factors (particularly G-CSF). CONCLUSION: Significant progress has been made in recent years in the field of idiosyncratic drug-induced neutropenia, leading to an improvement in their prognosis (currently, mortality rate between 5 and 10%). Clinicians must continue their efforts to improve their knowledge of these adverse events with new drugs as biotherapies.

Systematic Review and Pragmatic Clinical Approach to Oral and Nasal Vitamin B12 (Cobalamin) Treatment in Patients with Vitamin B12 Deficiency Related to Gastrointestinal Disorders.

The objective of this review is to provide an update on the effectiveness of oral and nasal vitamin B12 (cobalamin) treatment in gastrointestinal (GI) disorders. Relevant articles were identified by PubMed and Google Scholar systematic search, from January 2010 and June 2018, and through hand search of relevant reference articles. Additional studies were obtained from references of identified studies, the Cochrane Library and the ISI Web of Knowledge. Data gleaned from reference textbooks and international meetings were also used, as was information gleaned from commercial sites on the web and data from CARE B12 research group. For oral vitamin B12 treatment, 4 randomized controlled trials (vs. intramuscular), 4 narrative and 4 systematic reviews, and 13 prospective studies fulfilled our inclusion criteria. These studies concerned patients with vitamin B12 deficiency related to: food-cobalamin malabsorption (n = 6), Biermer's disease (n = 3), veganism or vegetarianism (n = 1), total gastrectomy after Roux-en-Y gastric bypass (n = 2) and Crohn's disease (n = 1). Four prospective studies include patients with vitamin B12 deficiency related to the aforementioned etiologies, except veganism or vegetarianism. The systematic present review documents that oral vitamin B12 replacement, at a daily dose of 1000 µg (1 mg), was adequate to normalize serum vitamin B12 levels and cure main clinical manifestations related to vitamin B12 deficiency, in GI disorders, and thus, with safety profile. For nasal vitamin B12 treatment, only one preliminary study was available. We conclude that oral vitamin B12 is an effective alternative to intramuscular vitamin B12 (except in patients presenting with severe neurological manifestations). Oral vitamin B12 treatment avoids the discomfort, contraindication (in patients with anticoagulation), and cost of monthly injections.