A Rare Coexistence of Multiple Myeloma and Polycythemia Vera.

Multiple myeloma (MM) is classically associated with organ dysfunction leading to hypercalcemia, renal insufficiency, anemia and bone disease, known as the CRAB criteria. More than 70% of patients with MM present with anemia. Few rare case reports, however, have demonstrated the presentation of MM associated with polycythemia. We present an interesting case of a 65-year-old female who was initially diagnosed with monoclonal gammopathy of undetermined significance (MGUS) which progressed to smoldering myeloma and later developed into MM. The patient also had coexisting polycythemia vera (PCV). We discuss the typical patient presentations as well as the expanded diagnostic criteria for MM. The pathophysiology explaining the coexistence of polycythemia and MM will be explored as well.

Sepsis-Related Outcomes of Patients with Philadelphia-Negative Myeloproliferative Neoplasms.

We analyzed the National Inpatient Sample (NIS) database to study the sepsis-related outcomes in patients with Philadelphia negative myeloproliferative neoplasms (MPN). A total of 82,087 patients were included, most had essential thrombocytosis (83.7%), followed by polycythemia vera (13.7%), and primary myelofibrosis (2.6%). Sepsis was diagnosed in 15,789 (19.2%) patients and their mortality rate was higher than nonseptic patients (7.5% vs 1.8%; p < .001). Sepsis was the most significant risk factor of mortality (aOR, 3.84; 95% CI, 3.51-4.21), others included liver disease (aOR, 2.42; 95% CI, 2.11-2.78), pulmonary embolism (aOR, 2.26; 95% CI, 1.83-2.80), cerebrovascular disease (aOR, 2.05; 95% CI, 1.81-2.33), and myocardial infarction (aOR, 1.73; 95% CI, 1.52-1.96).

Sepsis-related outcomes of patients with Philadelphia-negative myeloproliferative neoplasms.

We analyzed the National Inpatient Sample (NIS) database to study the sepsis-related outcomes in patients with Philadelphia negative myeloproliferative neoplasms (MPN). A total of 82,087 patients were included, most had essential thrombocytosis (83.7%), followed by polycythemia vera (13.7%), and primary myelofibrosis (2.6%). Sepsis was diagnosed in 15,789 (19.2%) patients and their mortality rate was higher than non-septic patients (7.5% vs 1.8%; P<.001). Sepsis was the most significant risk factor of mortality (aOR, 3.84; 95% CI, 3.51-4.21), others included liver disease (aOR, 2.42; 95% CI, 2.11-2.78), pulmonary embolism (aOR, 2.26; 95% CI, 1.83-2.80), cerebrovascular disease (aOR, 2.05; 95% CI, 1.81-2.33), and myocardial infarction (aOR, 1.73; 95% CI, 1.52-1.96).

Solitary Cystic Psoas Muscle Metastasis From Rectosigmoid Adenocarcinoma.

The most common subtype of colon cancer is colorectal adenocarcinoma. Compared with other subtypes, such as signet-ring and mucinous, colorectal adenocarcinoma has been found to have lower rates of metastasis. Approximately 20% of colorectal cancer cases present with metastatic disease on initial evaluation. The most common locations for metastasis are the liver, lung, peritoneum, bone, and extra-regional lymph nodes. Metastatic disease to the skeletal muscle, however, is considerably rare. We present a clinical case of a 52-year-old female found to have a cystic iliopsoas muscle metastasis from rectosigmoid adenocarcinoma, initially classified as an infected fluid collection.

Hereditary persistence of hemoglobin F is protective against red cell sickling. A case report and brief review.

Fetal hemoglobin (HbF) is a physiologic protein tetramer that is crucial for a developing fetus to survive in utero. Maternal hemoglobin has a relatively lower affinity for oxygen, and thus allows for an efficient transfer of oxygen from maternal to fetal blood. In addition to fulfilling a critical physiologic role, HbF is also known to alleviate symptoms of sickle-cell disease (SCD). The concentration of HbF depends on several factors. HbF is elevated in inherited conditions, such as hereditary persistence of HbF, hereditary spherocytosis, and thalassemia. The level of HbF is also increased in acquired states, such as pregnancy, aplastic anemia, thyrotoxicosis, hepatoma, myeloproliferative disorders, or hypoplastic myelodysplastic syndrome. It has been identified that some genetic loci have significant influence on HbF levels. The XmnI polymorphism, the HMIP locus, and the BCL11A gene are responsible for 45% of variations in HbF levels. Although SCD has been well described in the subpopulations of Africa, it is less common in the subpopulations of India. We describe a case of SCD, in which a patient with high HbF level presented at a very late age (27 years old). We presume the patient's inherently elevated HbF levels were able to compensate for the hypoxic episodes associated with SCD. The onset of symptoms was delayed as a result of elevated HbF levels.

Case of Donath-Landsteiner haemolytic anaemia in an adult female.

Donath-Landsteiner haemolytic anaemia (DLHA), also known as paroxysmal cold haemoglobinuria, is a very rare and difficult condition to diagnose as well as treat. Here, we present a case of a 55-year-old Hispanic woman who presented with severe intravascular haemolytic anaemia in the setting of a viral illness 2 weeks prior to presentation. Direct antiglobulin testing revealed mixed results: positive for either complement, IgG or both on various occasions which led to a battery of tests including the Donath-Landsteiner antibody testing which turned out positive establishing the diagnosis of DLHA. She was initially treated unsuccessfully with supportive care in the form of packed red blood cell transfusions and steroids as well as rituximab for about 4 weeks but her condition improved on cyclophosphamide, and she is on the road to recovery after 10 weeks of hospital stay.

Primary peritoneal carcinoma metastasizing to breast: a single case report and literature review from clinic to biology.

Primary peritoneal carcinoma (PPC) is a type of rare malignant epithelial tumor. Metastasis from PPC to breast has been rarely reported. PPC originates de novo from the peritoneal tissues rather than invasion or metastasis from adjacent or remote organs. PPCs have been implicated in many cases of carcinomas of unknown primary origin. It is similar to ovarian cancer (OvCa), because it shares the same common embryonic origin, the coelomic epithelium (mesodermal origin). The mechanism of oncogenesis remains elusive. In this article, we report a rare case of PPC in a patient 10 years after total abdominal hysterectomy and bilateral salpingooophorectomy for uterine leiomyoma, which was widely spread in the abdomen and metastasized to the colon, liver and distant organs including breast. The treatment is similar to that of primary ovarian cancer. We also reviewed the primary peritoneal cancer metastatic to breast and discuss the possible mechanisms and biology of primary peritoneal cancer, using experimental and animal model.