[Blastic plasmacytoid dendritic cell neoplasm (BPDCN) : A rare hematologic neoplasm with frequent cutaneous involvement]. / Blastische plasmazytoide dendritische Zellneoplasie (BPDCN) : Eine seltene hämatologische Neoplasie mit häufiger kutaner Beteiligung.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy that derives from precursors of plasmacytoid dendritic cells and is characterized by disseminated, erythematous or bluish-livid plaques or nodi. Because of the disease's rarity the diagnosis and treatment still pose a significant challenge. We present a case of a patient with BPDCN and show clinical and diagnostic characteristics as well as potential treatment regimes.

Thrombocytopenia in Children: A Large Prospective Study on Clinical Manifestations, Seasonal Variation, Etiology, and Outcome.

AIM: To study the clinico-etiological profile of children with thrombocytopeniaMethods: This prospective hospital-based study included all children (<18 years) with thrombocytopenia at the time of hospitalization and/or thrombocytopenia during the course of their hospital stay. A detailed history was recorded and appropriate laboratory investigations were carried out Results: The study group comprised 246 children (mean age, 9.29 years; median age, 10 years) with male to female ratio of 1.5:1. Nearly 45% of children were above 10 years of age. Trends of admissions showed that the majority of children with thrombocytopenia (n = 115) got hospitalized during the rainy season, followed by summer (n = 84). Fever (72.8%), pallor (52.8%), bleeding manifestations (22%), lymphadenopathy (20.3%), and splenomegaly (20.3%) were common clinical features. Petechiae was the most common bleeding manifestation (63%). Septicemia (24%) was the most common etiology, followed by megaloblastic anemia (14.6%), undiagnosed fever (10.2%), local infection (9.3%), hepatitis (6.5%), and scrub typhus (6.1%). About nine children died. All those who died had septicemia and multi-organ dysfunction (MOD). On logistic regression analysis, age >10 years, presence of bleeding, arthralgia, rash, pallor, gastrointestinal (GI) symptoms, hematological disorders, and malignancy were associated with severe thrombocytopeniaConclusion: Thrombocytopenia is a common hematological observation. This study revealed seasonal variation in the occurrence of thrombocytopenia in children, with the maximum number of cases in the rainy season. Septicemia is the commonest etiology. The majority of children with thrombocytopenia have no bleeding manifestations. Age >10 years, presence of bleeding, arthralgia, rash, pallor, GI symptoms, hematological disorders, and malignancy are associated with severe thrombocytopenia.

Pancytopenia in celiac disease-A case series of 20 children.

Pancytopenia in children with celiac disease (CeD) is postulated to be due to nutritional deficiency such as vitamin B12, folate and copper or an autoimmune process resulting in aplastic anemia with hypoplastic marrow. In the present case series, we report the profile and explore the etiology of pancytopenia among children with CeD. There are only a few case reports of pancytopenia in children with CeD. We enrolled newly diagnosed cases of CeD and pancytopenia presenting in the celiac disease clinic over three years. Detailed evaluation was carried out for the cause of pancytopenia. We followed up on the cases for compliance and response to gluten-free diet at three months, six months and 12 months. Twenty patients were eligible for inclusion. They were divided into two groups: one with aplastic anemia with hypoplastic marrow labeled as Gp CeD-AA and the other with megaloblastic/nutritional anemia labeled as Gp CeD-MA. Patients in Gp CeD-MA presented with classical symptoms of CeD as recurrent diarrhea, abdomen distension, pallor and poor weight gain. They had none or just one transfusion requirement and had an early and complete recovery from pancytopenia. Patients in Gp CeD-AA presented with atypical symptoms such as epistaxis, short stature, fever, pallor and weakness. They had a multiple blood transfusion requirement and had delayed and partial recovery from pancytopenia. Pancytopenia is not a disease in itself but is the presentation of an underlying disease. It can occur due to various coexisting disorders in children with CeD, which can be as simple as nutritional deficiencies to as complex as an autoimmune process or malignancy. CeD should be included in the differential diagnosis of aplastic anemia as CeD and aplastic anemia both have a similar pathological process involving T cell destruction of tissues.

Atypical Optic Neuritis: The Potential Red Flags.

Background: Many potential causes of optic nerve inflammation exist, including typical and atypical causes, which require different management strategies. Objective: The objective of this study is to identify red flags that help differentiate typical from atypical optic neuritis (ON). Materials and Methods: This prospective study included 66 patients (100 eyes) with immune-mediated ON from January 2016 to June 2019, carefully excluding the nonimmune causes. The clinico-radiological features, investigations, therapy, and outcome were analyzed. Results: We evaluated 33 cases each of typical and atypical ON. The typical group included 29 idiopathic ON and four associated with multiple sclerosis. Atypical ON included 19 neuromyelitis optica (NMO), seven MOG-associated ON (MOG-ON), and others due to Sjogren's syndrome, granulomatous polyangiitis, sarcoidosis, and IgG4 disease. Atypical ON occurred significantly and more frequently with extremes of ages (<10 or >70 years), bilateral simultaneous or severe vision loss with early disc pallor, multiple attacks, symptoms/neuro-imaging indicating non-MS disease e.g., long segment ON/myelitis, large confluent lesions, the involvement of optic tract, chiasma, area postrema or diencephalon, and (pachy) meningitis. Systemic involvement and poor outcomes despite steroids and second-line immunosuppression were observed more often in the atypical ON. Conclusions: The red flags indicating atypical ON are onset at extremes of age, multiple attacks, bilateral simultaneous or severe to very severe vision loss, early disc pallor, neurological symptoms, or imaging abnormalities suggesting non-MS disease, systemic involvement, and poor steroid responsiveness. The awareness might help the clinician promptly identify and escalate therapy to ensure a better outcome.

True Idiopathic Brachial Artery Aneurysm Treated With a Saphenous Vein Graft.

Purpose: The objective of this study is to report a case of a 65-year-old woman who presented with pallor and pain of her left arm secondary to a true arterial brachial aneurysm, which was successfully treated with saphenous vein bypass and embolization of the aneurysm sac. A review of the literature is also presented. Case report: A 65-year-old woman presented with an acute onset of pallor and pain of her left forearm, and hand. On physical examination, there was a pulsatile mass at the forearm. A doppler ultrasound showed a fusiform aneurysmal dilatation of the brachial artery of 23 mm of diameter. A dynamic contrast-enhanced MRI angiogram confirmed a fusiform dilation of the distal brachial artery. The patient was scheduled for open repair. A fusiform 20 x 60 mm aneurysm of the distal brachial artery extending to the cubital fossa was found and a brachial artery to radial and ulnar arteries bypass with interposed reverse right saphenous vein was created. Embolization of the aneurysm sac was performed using Gelita-spon ® (Gelita Medical, Eberbach, Germany). A final angiogram showed an adequate perfusion through the bypass to the hand, and no contrast in the aneurysmal sac. Postoperative course was uneventful with discharge on the fourth postoperative day. Conclusion: Revascularization with autologous saphenous vein graft and exclusion of the aneurysm with local embolization is a good treatment alternative in a patient with symptomatic brachial aneurysm with distal embolization.