Unprovoked Pulmonary Embolism in Factor VII Deficiency.

Thrombotic events in bleeding disorders such as hemophilia A or B, Von Willebrand disease, afibrinogenemia, factor VII deficiency, and factor XI deficiency are rare but have been reported. These events usually occur in the presence of prothrombotic risk factors such as recent surgery, trauma, or factor replacement therapy. We present a case of a 68-year-old Hispanic female with a history of factor VII deficiency who presented with shortness of breath, chest pain, and palpitations and was found to have pulmonary embolism. Our patient did not have any of the above-mentioned thrombotic risk factors. Our case and review of the literature show that factor VII deficiency does not provide protection against thrombosis.

Atypical Presentation of Thrombotic Thrombocytopenic Purpura without Hematological Features.

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease, usually diagnosed with high index of suspicion. The pathophysiology of TTP is due to severe deficiency of von Willebrand factor cleaving protease, known as ADAMTS 13. Early diagnosis is crucial as without treatment TTP is associated with high mortality rate. Plasma exchange is currently the mainstay of treatment. Nonetheless, the classical pentad of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, neurological dysfunction, kidney dysfunction and fever are seen only in 40 percent of the patients. MAHA and thrombocytopenia are the common presenting features. Presentation with thrombotic complication without hematological features (MAHA and thrombocytopenia) is rare and makes the diagnosis difficult. Herein, we report an unusual presentation of a 53-year-old male, who was initially presented in 2014 with classical features of TTP, however had an atypical presentation of TTP in 2016 with only neurological features without hematological features.

A rare and unusual presentation of Epstein-Barr virus-associated diffuse large B-cell lymphoma involving colon as the primary site.

Lymphoproliferative malignancies can involve both nodal- and extra-nodal tissues. The most common extranodal site involved is the gastrointestinal (GI) tract, and it is secondary to the widespread primary nodal disease. However, about 33% of non-Hodgkin's lymphoma primarily arise from tissues other than lymph nodes, spleen, or bone marrow, for example, GI tract, skin, or the central nervous system and are called primary extranodal lymphomas. The most common site of GI localization is stomach (50%-60%) followed by small bowel. Primary colonic lymphoma is seen only in 6% of GI lymphomas and up to 0.5%-1% of all colon malignancies. Hence, primary GI lymphoma is extremely rare, and primary colonic lymphoma is an even rarer occurrence. There is clearly a paucity of cases reported in literature resulting in unclear treatment protocol. Here, we report a case of a 51-year-old man who presented with abdominal pain, weight loss, and bright red blood per rectum. A colonoscopy revealed diffuse bleeding ulcers involving the entire colon. Pathology was consistent with primary diffuse large B-cell lymphoma arising from the colon. The patient was started on treatment with rituximab, cyclophosphamide, adriamycin, vincristine, and prednisone.

Severe Metabolic Acidemia in a Patient with Aleukemic Leukemia.

Malignancy associated lactic acidosis is a rare metabolic complication that may accompany various types of malignancies. To date, most cases that have been reported are associated with hematologic malignancies (lymphoma and leukemia). Many theories have been proposed to explain the pathophysiology of lactic acidosis in malignancies. We are reporting an unusual case of a 62-year-old female who presented with a complaint of generalized weakness. Patient was found to have pancytopenia and metabolic acidosis with an anion gap secondary to lactic acid in addition to non-anion gap acidosis (NAGA). The lactic acidosis resolved only after initiation of chemotherapy as she was diagnosed with B-cell acute lymphoblastic leukemia. Our patient also had a coexistent Renal Tubular Acidosis (RTA) with large kidneys. The kidney size also decreased with chemotherapy. Our case is unique as evidenced by aleukemic leukemia combined with anion gap acidosis and non-anion gap acidosis. Lactic acidosis has many different causes; although rare, hematologic malignancies should be included in the differential diagnosis regardless of cell counts or tumor burden.

The Role of Red Cell Distribution Width as a Predictor of Mortality for Critically Ill Patients in an Inner-city Hospital.

BACKGROUND: Red cell distribution width (RDW) is a measure of the variation in the red blood cell volume that is usually recorded as a part of the standard complete blood cell count. Recent studies have demonstrated the prognostic value of RDW in many different clinical settings. The objective of this research study is to investigate the independent association of RDW with 30-day mortality in Intensive Care Unit (ICU) patients. METHODS: One hundred and fifty-six patients admitted to the ICU of our hospital between July 2009 and June 2011 were included in our study. Out of 156 patients, 124 survived the hospital stay. The data on patient's demographics, interventions done in ICU, and their comorbidities were collected. Baseline variables and the RDW value were compared between survivors and nonsurvivors. The cutoff point for RDW used for the comparison was 15.75. Both univariable and multivariable analyses were done. P < 0.05 was considered statistically significant. RESULTS: In the univariable analysis of the study between survivors and nonsurvivors, the median RDW was 17.20 for nonsurvivors, implying statistical significance (P = 0.007). In multivariable analysis, RDW remained significantly associated with inpatient mortality. The receiver operating characteristic is 0.656 (P = 0.007), with an optimal cutoff of 15.75 for RDW. At the cutoff of RDW, i.e., 15.75, the sensitivity and specificity for inpatient mortality was 71% and 89%, respectively. CONCLUSION: In critically ill ICU patients, RDW is an independent predictor of 30-day mortality. Taking into consideration the fact that RDW is routinely measured in complete blood count with no additional cost, this can serve as an "inexpensive prognostic marker" in critically ill patients.