Sweet syndrome associated with differentiation syndrome in a patient with acute promyelocytic leukemia – a case report and review of literature
Sweet Syndrome (SS) (acute febrile neutrophilic dermatosis) is an inflammatory disorder characterized by the appearance of painful, edematous, and erythematous papules, plaques, or nodules on the skin. It might also cause fever and internal organs’ involvement. There are three clinical settings in which SS has been described: classical or idiopathic, malignancy-associ¬ated and drug-induced. (1) SS is described in patients with acute promyelocytic leukemia (APL), and is a rare side effect of all-trans retinoic acid (ATRA), which causes differentiation of promyelocytes and used in therapy of APL. ATRA may also cause Differentiation Syndrome (DS), previously called retinoic acid syndrome, and there is overlap with SS. (2) There is a limited number of case reports describing SS in association with DS. We present here a case of SS associated with DS in a patient with APL treated with ATRA.
Goblet Cell Carcinoid (GCC) of the Appendix presenting as a Small Bowel Obstruction: Case Report and Literature Review
Goblet Cell Carcinoid (GCC) is a rare, low grade malignancy, and GCC presenting as a small bowel obstruction (SBO) is incredibly rare. Hereby, we presenting a 68-year-old male presented to our Emergency Department with a one-day history of right sided abdominal pain, distention and vomiting, he had no previous abdominal surgery and had no significant other medical problem. Abdominal Computed Tomography (CT) demonstrated a high grade, closed loop small bowel obstruction involving the terminal segment of the ileum, and a low-density appendiceal nodule. He had laparoscopy converted to open caecectomy, the histology, confirmed the diagnosis of Goblet Cell Carcinoid of the appendix, eventually he had right hemicolectomy, progressed well, and made a good post-operative recovery, discharged home. As presentation of GCC of the appendix with sbo is a very rare incidence, we discussed this through a case report with its immunohistochemical, behavioral features, presentation and treatment options.
T Prolymphocytic Leukemia, a rare disease, case presentation with typical pathological findings and review of management
Background: Prolymphocytic leukemia (PLL) is a rare disease, accounting for < 2% of lymphoid leukemias .There are 2 quite different entities with distinct pathologic features and different therapeutic strategies, T-cell and B-cell . T-PLL is more common . Although termed ‘prolymphocytic,’ T-PLL is characterized by the proliferation of post-thymic T-lymphocytes. It is estimated that physicians will see a case of T-PLL every five to ten years . Over the last 15 years, 3 cases of T-PLL were diagnosed in our center. We present here the latest case, with typical pathological findings. Case Report: A 63-year-old British gentleman, previously healthy with good performance, presented with fatigue for 6 – 8 weeks with puffiness of face and eyelids and weight loss of 5 kilograms. Physical exam showed periorbital edema and conjunctival injection (Figure 1), cervical lymphadenopathy, and hepatosplenomegaly. Initial complete blood count (CBC): white blood cells (WBCs) 331.9 x109/L (4.0 – 10.0), with 94% lymphocytes, hemoglobin (Hb) 119 gm/L (130 – 170), platelets (Plts) 128 x109/L (150 – 400). Lactate dehydrogenase 10.4 µkat/L (2.1 – 3.7), uric acid 537 µmol/L (210 – 420). Abdomen ultrasound showed markedly enlarged spleen (20.3 cm). The peripheral blood smear showed profound leukocytosis with 94% abnormal lymphoid cells. The cells were small to medium in size with high nucleo-cytoplasmic ratio, moderately condensed chromatin and almost all with prominent nucleolus. Substantial number of the cells show irregular nuclear contour with short indentation. The cytoplasm was basophilic agranular and many cells show irregular cytoplasmic protrusions (Figure 2). Flow cytometry on peripheral blood revealed one homogenous abnormal population positive for cluster of differentiation 45 (CD45) and express the pan T-cell markers (CD3, CD2, CD5 and CD7). All were positive for CD4, T-cell receptor (TCR) alpha/beta, CD43 and BCl2 with partial expression of CD38. These cells were negative for CD56, CD57,...
Benzodiazepines have anxiolytic, hypnotic, anticonvulsant, and muscle-relaxing properties, therefore, a widely prescribed treatment for anxiety and insomnia. They bind to gamma-aminobutyric acid type A (GABAA) receptors, which are responsible for most of the inhibitory neurotransmission in the central nervous system and these receptors are a major target of alcohol, barbiturates, muscle relaxants, and other medications with sedative effects, resulting in tolerance and dependence . Benzodiazepines are categorized into short (15 to 30 minutes), intermediate (30 to 60 minutes), and long-acting agents (one hour or longer). Benzodiazepines are frequently prescribed for elderly patients living in the community and for those in hospitals and institutions. Prolonged use of benzodiazepines is particularly likely in old age for the treatment not only of insomnia and anxiety, but also of a wide range of nonspecific symptoms. Long term users are likely to have multiple concomitant physical and psychological health problems . Benzodiazepines produce dependence, reduce attention, memory, and motor ability. They can cause disinhibition or aggressive behavior, facilitate the appearance of delirium, and increase accident and mortality rates in people older than 60 . According to Maudsley guidelines, benzodiazepines use in elderly are poorly supported for their link to cognitive decline, increase risk of falls and hip fractures . And, if indicated, short acting agents should be avoided. Long acting agents should be started in low doses and patients need to be followed and reassessed regularly. Zolpidem or melatonin are indicated for insomnia, clonazepam and diazepam for agitation and pregabalin for generalized anxiety disorder .
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy that is characterized by intravascular hemolytic anemia, thrombocytopenia and acute renal failure. Typical form occurred with the shiga-like toxin produced by Escherichia coli bacterium or Shigella dysenteriae bacterium while atypical HUS is sporadic or familial form of the disease which develops due to uncontrolled activation in alternative complement pathway. It may present with recurrent attacks and it has a potential of poor prognosis. In this case report we presented a 36-year-old woman in her 22nd week pregnancy without any history of chronic disease or medication whom admitted to hospital with abdominal pain, bloody diarrhea, oliguria and diagnosed with atypical HUS. She was diagnosed with ileus at the first month of Eculizumab treatment and a polyploid lesion obstructing the lumen of the colon was detected. Pathologic examination of the mass revealed a chronic active inflammatory lesion.
Ergotamine and its derivatives were used for multiple diseases; in the nineties, its use in association with caffeine was considered a standard treatment for migraines, although it has been in decline, due to the evidenced toxic effects related to its chronic use including vascular, cardiovascular and central nervous system manifestations. In this article is reported a case of a 59-year-old female with chronic use of caffeine plus ergotamine for more than 20 years; afterwards she develops peripheral artery disease, severe mitral and tricuspid valve regurgitation with requirement of valve replacement. In the absence of past cardiovascular history and rheumatic fever, its clinical evolution and pathology results are associated with ergotism.
Paraparesis following commencement of Anti Tuberculosis drugs for latent Tuberculosis. Case report and review of literature
A 43 year old female Patient, with recently diagnosed latent Tuberculosis (TB), started On anti – TB drugs (Isoniazid, Rifampicin), came with symptoms of Motor and sensory polyneuropathy only 4 weeks after starting the drugs. Extensive investigations were pursued. Nerve conduction study showed Features of Guillain – Barre syndrome (GBS). After stopping the Anti TB medications, patient symptoms Improved. This gives us an idea that Anti TB medications can itself trigger GBS. The Association of TB and GBS will be reviewed in Literature as well as the differentials and management.
Chronic myeloid leukemia (CML) is a Philadelphia (Ph) chromosome-positive myeloproliferative neoplasm. The molecular consequence of reciprocal translocation t(9;22)(q34;q11) is the generation of the BCR–ABL fusion gene, which encodes a constitutively active tyrosine kinase signaling protein. The tyrosine kinase is responsible for the leukemia phenotype through the constitutive activation of multiple signaling pathways involved in the cell cycle and in adhesion and apoptosis. Dasatinib is an oral BCR-ABL tyrosine kinase inhibitor (TKI) which is mainly used for treating CML patients with resistance or intolerance to Imatinib. Dasatinib has several significant adverse reactions and gastrointestinal side effects including colitis. We report a case of a 33-year-old male, diagnosed with CML (Chronic Phase) who received Dasatinib as second-line therapy and developed chronic diarrhea and colitis attributed to Dasatinib and improved after stopping it.
Diagnosis of ovarian tumor during pregnancy presents a management dilemma. We herein report a case of huge ovarian cyst adenoma complicating pregnancy in a 25 years old gravida 3, previous two caesarian, presenting at 13 wks. pregnancy. She was referred from periphery for uterine size more than the period of gestation and ultrasound (USG) showing 25×12 cm ovarian cyst. USG was inconclusive of benign or borderline ovarian tumor. Neoadjuvent chemotherapy followed by cytoreductive surgery was planned. USG guided fine needle aspiration cytology (FNAC) was done before starting chemotherapy which suggested benign etiology. Management plan was changed to continuation of pregnancy and conservative surgery at 20 wks of pregnancy. Planned exploratory laparotomy with left sided salpingoophorectomy was done under general anesthesia at 22 wks. of gestation. Post-operative period was uneventful. Patient was delivered by LSCS at 38 wks. of gestation.
Anomalous Right Coronary Artery with LV Non-compaction having Malignant Course, A Rare but Lethal Combination
Anomalous Right coronary Artery origin from contralateral sinus is a rare but lethal variant that may mimic like acute coronary syndrome and is a potential cause of sudden cardiac death. This needs early diagnosis with prompt treatment which may be conservative or interventional depending upon the clinical presentation, exact anatomy and route of the vessel. Here we present a unique case of combination of anomalous right coronary artery origin from opposite sinus causing ischemia, with LV non-compaction ending in non-hemorrhagic stroke.