Resolution of cardiac surgical bleeding with the combination of 4-factor prothrombin complex concentrate and fresh frozen plasma following lack of response to fresh frozen plasma alone in a patient with severe factor XI deficiency.

Factor XI deficiency is associated with a bleeding tendency in some patients. Factor XI helps to reduce fibrinolysis. Bleeding risk is increased in factor XI-deficient patients during surgeries with high fibrinolytic activity, including nasopharyngeal/oropharyngeal and genitourinary surgeries. Treatment options for factor XI-deficient patients include fresh frozen plasma (FFP), antifibrinolytics, recombinant factor VIIa, and factor XI concentrates (available in Australia, Canada, and some European countries). 4-factor prothrombin complex concentrate (4-factor PCC) is an extract of FFP comprised of unactivated factors II, VII, IX, and X, proteins C and S, and heparin. It has been used for cardiac surgical bleeding. We report the first case of a patient with severe factor XI deficiency and cardiac surgical bleeding, which resolved with the combination of 4-factor PCC and FFP after lack of response to FFP alone.

Myelomatous Pleural Effusion Presenting with Extreme Hyperferritinemia and Severe Inflammatory Response.

Myelomatous involvement of pleural effusions developing in patients with multiple myeloma is extremely rare and only a few cases have been reported so far. It is thought to represent an aggressive clinical progression of disease and is usually associated with severe complications, poor prognosis and high mortality. Ferritin is a marker of inflammatory pathways that plays a significant role in plasma cell malignancies and has been studied as a prognostic factor for multiple myeloma. In severe inflammatory states such as septic shock or hemophagocytic lymphohistiocytosis, extreme levels of ferritin are thought to precipitate a cytokine storm associated with poor clinical outcomes. We present a case of myelomatous pleural effusion associated with extreme levels of ferritin and explore the possibility of a connection between this rare entity and other severe inflammatory states, which could account for its ominous outcomes and poor prognosis.

Clinical and cytogenetic characteristics of myelodysplastic syndrome in patients with HIV infection.

We report eight patients of myelodysplastic syndrome (MDS) with HIV infection. Compared to a historical cohort of HIV-uninfected MDS patients, HIV/MDS were younger (p=0.019), had more complex cytogenetics (p=0.015), and more often had 7q deletion or monosomy 7 (p=0.011). In five patients, HIV/MDS transformed to acute myeloid leukemia, with a median time to transformation of 7 months. Also, the median overall survival was shorter in the HIV/MDS than in their HIV-uninfected counterparts (8 vs. 22 months; p=0.003). These results suggest that HIV/MDS is a high-risk MDS necessitating thorough cytogenetic analysis and follow-up.

Heparin-induced thrombocytopenia: analysis of risk factors in medical inpatients.

Heparin-induced thrombocytopenia (HIT) is an unpredictable reaction to heparin characterized by thrombocytopenia and increased risk of life-threatening venous and/or arterial thrombosis. Data are lacking regarding additional risk factors that may be associated with the development of HIT. This study aimed to identify the risk factors that may be associated with HIT in medical inpatients receiving heparin. Twenty five thousand six hundred and fifty-three patients admitted to the medicine service who received heparin product were reviewed retrospectively. The diagnosis of HIT was confirmed if the platelet count dropped >50% from baseline and there was a positive laboratory HIT assay. Fifty-five cases of in-hospital HIT were observed. Multivariate analysis identified the administration of full anticoagulation dose with unfractionated heparin or exposure to heparin products for more than 5 d with an increased risk of HIT. Moreover, patients who were on haemodialysis, carried a diagnosis of autoimmune disease, gout or heart failure were also at increased risk. The results suggest that when using heparin products in these patient cohorts, increased surveillance for HIT is necessary.

Unilateral serous retinal detachment in a patient with thrombotic thrombocytopenic purpura.

Ocular complications are a relatively common occurrence in patients with thrombotic thrombocytopenic purpura (TTP). Serous retinal detachment is a rare but reported complication, with most cases being bilateral and associated with concomitant hypertension. Therapeutic plasma exchange has been used to successfully treat patients with TTP. We report a case of a 46 year old woman who presented with TTP, received therapeutic plasma exchange, and developed unilateral serous retinal detachment with retinal pigment epithelial tear in the absence of reported hypertension. The increased perfusion pressure due to hypertension, combined with the choroidal vasculature damage from TTP are thought to lead to retinal epithelial tear. This suggests that although hypertension may play a role in retinal detachment in these patients, other mechanisms may be responsible.

Non-Hodgkin's lymphoma of mucosa-associated lymphoid tissue.

The concept of mucosa-associated lymphoid tissue (MALT) lymphomas was introduced by Isaacson and Wright [Cancer 1983; 52:1410-1416] in 1983. After more than 20 years of clinical research MALT lymphomas are now recognized as a distinct subtype of non-Hodgkin's lymphoma (NHL) with unique pathogenic, histological, and clinical features. Although this subtype of NHL occurs frequently, optimal management remains elusive. This manuscript reviews features of the clinical presentation, diagnosis, pathology, molecular characteristics, and management of both gastric and non-gastric MALT lymphoma.

Management of adult idiopathic thrombocytopenic purpura.

Idiopathic thrombocytopenic purpura (ITP) is defined as isolated thrombocytopenia without a clinically apparent cause. It is categorized as acute, chronic, and refractory. Its clinical presentation ranges from acute to insidious and the bleeding may vary from minimal to severe. The target platelet count with therapy is more than 30,000/microL in sedentary individuals. Since studies regarding therapies for ITP have been mostly uncontrolled case series, the treatment recommendations are largely derived from expert opinion. This review paper summarizes the data on available therapies for adult acute and chronic/refractory ITP. The therapies include splenectomy, steroids, intravenous immunoglobulin, anti-Rh(D), monoclonal antibodies, danazol, chemotherapy, plasma exchange, and others.

Simultaneous appearance of trisomy 8 and trisomy 12 in different cell populations in a patient with untreated B-cell chronic lymphocytic leukemia and myelodysplasia.

The co-existence of spontaneously arising myeloid and lymphoid malignancies in the same patient is rare, and is thought to be mainly due to chance. We describe a patient presenting simultaneously with chronic lymphocytic leukemia (CLL) and myelodysplasia (MDS). Histological, flow cytometric, chromosomal and fluorescent in situ hybridization (FISH) studies show that both cell populations possess different sets of markers consistent with the myeloid and lymphoid differentiation pathways. The question of whether these arose from a single or two separate progenitor cells is explored.

Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment.

CONTEXT: Hydroxyurea increases levels of fetal hemoglobin (HbF) and decreases morbidity from vaso-occlusive complications in patients with sickle cell anemia (SCA). High HbF levels reduce morbidity and mortality. OBJECTIVE: To determine whether hydroxyurea attenuates mortality in patients with SCA. DESIGN: Long-term observational follow-up study of mortality in patients with SCA who originally participated in the randomized, double-blind, placebo-controlled Multicenter Study of Hydroxyurea in Sickle Cell Anemia (MSH), conducted in 1992-1995, to determine if hydroxyurea reduces vaso-occlusive events. In the MSH Patients' Follow-up, conducted in 1996-2001, patients could continue, stop, or start hydroxyurea. Data were collected during the trial and in the follow-up period. SETTING: Inpatients and outpatients in 21 sickle cell referral centers in the United States and Canada. PATIENTS: Two-hundred ninety-nine adult patients with frequent painful episodes enrolled in the follow-up. Follow-up data through May 2001 were complete for 233 patients. INTERVENTION: In the MSH, patients were randomly assigned to receive hydroxyurea (n = 152) or placebo (n = 147). MAIN OUTCOME MEASURE: Mortality, HbF levels, painful episodes, acute chest syndrome, and blood cell counts. The randomized trial was not designed to detect specified differences in mortality. RESULTS: Seventy-five of the original 299 patients died, 28% from pulmonary disease. Patients with reticulocyte counts less than 250 000/mm3 and hemoglobin levels lower than 9 g/dL had increased mortality (P =.002). Cumulative mortality at 9 years was 28% when HbF levels were lower than 0.5 g/dL after the trial was completed compared with 15% when HbF levels were 0.5 g/dL or higher (P =.03 ). Individuals who had acute chest syndrome during the trial had 32% mortality compared with 18% of individuals without acute chest syndrome (P =.02). Patients with 3 or more painful episodes per year during the trial had 27% mortality compared with 17% of patients with less frequent episodes (P =.06). Taking hydroxyurea was associated with a 40% reduction in mortality (P =.04) in this observational follow-up with self-selected treatment. There were 3 cases of cancer, 1 fatal. CONCLUSIONS: Adult patients taking hydroxyurea for frequent painful sickle cell episodes appear to have reduced mortality after 9 of years follow-up. Survival was related to HbF levels and frequency of vaso-occlusive events. Whether indications for hydroxyurea treatment should be expanded is unknown.