Endocarditis with Streptococcus pseudoporcinus Associated with Mastocytosis and Spondylodiscitis-A Coincidental Association? A Case Report.

Streptococcus pseudoporcinus is a nonmotile Gram-positive, catalase, and benzidine negative, arranged in short chains, isolated from the genitourinary tract group B Streptococcus. S. pseudoporcinus was also identified from blood, urine, skin, cervical area, wounds, rectum, and placenta samples. Two cases of infective endocarditis have been reported in the literature. Based on these data, the identification of a case of S. pseudoporcinus infective endocarditis associated with spondylodiscitis in a patient with undiagnosed systemic mastocytosis until the age of 63 years is unusual. Two sets of blood specimens were collected, and both sets were positive for S. pseudoporcinus. Transesophageal echocardiography revealed, multiple vegetations on the mitral valve. A lumbar spine MRI revealed L5-S1 spondylodiscitis that associates prevertebral and right paramedian epidural abscesses with compressive stenosis. The performed bone marrow biopsy, and cellularity examination revealed 5-10% mast cells in the areas of medullary tissue, an aspect that is suggestive of mastocytosis. Antibiotic therapy was initiated, under which the patient presented intermittent fever. A second transesophageal echocardiography revealed a mitral valve abscess. A mitral valve replacement with a mechanical heart valve device through a minimally invasive approach was performed, with a favorable evolution under treatment. S. pseudoporcinus can be responsible for infectious endocarditis in certain immunodepressed cases, but also in a profibrotic, proatherogenic field, as shown by the association with mastocytosis in the presented case.

A case of Fitz Hugh Curtis syndrome mimicking an acute abdomen.

Fitz Hugh Curtis syndrome, also known as acute perihepatitis, associates pelvic inflammatory disease with the presence of Chlamydia trachomatis or Neisseria gonorrhoeae as the main causative pathogens. Symptomatology is a nonspecific one. Right upper quadrant pain, fever, nausea and vomiting are the most commonly encountered symptoms. Imaging data are also nonspecific and often show intra-abdominal changes with no particularity. As it is difficult to suspect Fitz Hugh Curtis syndrome upon first impression, laparoscopy and direct visualization of the peritoneum and liver adhesions are needed in the diagnostic process. The specific aspect of the fibrinous strands can raise the suspicion of this disease and guide the subsequent treatment. We present the case of a 19-year-old patient with abdominal pain observed in the right upper quadrant and moderate anemia for which she was investigated in the hematology ward. The unfavorable evolution with the appearance of anemia and peritonitic acute abdomen signs required a surgical approach. The intraoperative aspects raised the suspicion of Fitz Hugh Curtis syndrome. Because of the nonspecific clinical picture as well as the insignificant imaging features, this condition can be a diagnostic and therapeutic challenge.

MECOM, HBS1L-MYB, THRB-RARB, JAK2, and TERT polymorphisms defining the genetic predisposition to myeloproliferative neoplasms: A study on 939 patients.

Polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) are classical myeloproliferative neoplasms (MPN), characterized by specific somatic mutations in JAK2, CALR or MPL genes. JAK2 46/1 and TERT rs2736100 polymorphisms are known to significantly predispose to MPN. This study aimed to establish the additional contribution of the recently described MECOM rs2201862, HBS1L-MYB rs9376092 and THRB-RARB rs4858647 polymorphisms to the occurrence of MPN. These three polymorphisms, along with JAK2 46/1 and TERT rs2736100 were genotyped in 939 MPN patients (454 with ET, 337 with PV and 148 with PMF) and 483 controls. MECOM rs2201862 associated significantly with each MPN entity, except for ET, and with all major molecular sub-types, especially those CALR-mutated (OR = 1.4; 95% CI = 1.1-1.8; P-value = .005). HBS1L-MYB rs9376092 associated only with JAK2 V617F-mutated ET (OR = 1.4; 95% CI = 1.1-1.7; P-value = .003). THRB-RARB rs4858647 had a weak association with PMF only (OR = 1.5; 95% CI = 1-2.1; P-value = .04). Surprisingly, JAK2 46/1 haplotype was associated significantly not only with JAK2 V617F-mutated MPN, but also with CALR-mutated MPN (OR = 1.4; 95% CI = 1.1-1.8; P-value = .01). TERT rs2736100 was associated equally strong with all MPN, regardless of phenotype or molecular sub-type. In conclusion, JAK2 46/1, TERT rs2736100 and MECOM rs2201862 are the chief predisposing polymorphisms to MPN.