Search PubMed⌕ Search

Biomedical subjects

D P Steensma

Publications and source records attributed to D P Steensma.

18 recordsLinked to original sources
PubMed2001

A high bone marrow plasma cell labeling index in stable plateau-phase multiple myeloma is a marker for early disease progression and death.

The plasma cell labeling index (PCLI) is a measure of plasma cell proliferative activity and is an important prognostic factor in newly diagnosed multiple myeloma (MM). Occasionally patients have been observed with stable, plateau phase MM with minimal numbers of residual light-chain-restricted monoclonal plasma cells, but a high PCLI. No data are available on the outcomes for such patients. Data from 57 patients with plateau phase MM and a marrow PCLI of more than 1.0% were compared with 105 matched control patients with MM with a marrow PCLI of less than 1.0%. All patients had less than 10% total plasma cells on marrow aspirate and biopsy. The median time to progression and overall survival were 8 months and 20 months, respectively, in the high PCLI group versus 39 months and 56 months, respectively, in the low PCLI group (P < .0001). These findings suggest that a high PCLI in patients with apparently stable, plateau phase MM is an adverse parameter that may predict a short time to disease progression and death.

Bone Marrow↗
PubMed2001

The narrow path.

Explore the source record for details and available documents.

Female↗
PubMed2001

Amifostine alone and in combination with erythropoietin for the treatment of favorable myelodysplastic syndrome.

Ten anemic patients with favorable myelodysplastic syndrome (MDS) were first treated with two 5-week courses of amifostine alone (each course consisted of 200 mg/m(2) of the drug given intravenously three times a week for 3 weeks), followed by an additional two courses combined with subcutaneous erythropoietin (EPO) (150 U/kg, three times a week for 8 weeks). The study patients either had previously failed to respond to treatment with EPO or had pretreatment serum EPO levels of more than 100 mU/ml. None of the patients experienced a complete or partial response in anemia or other cytopenias. We conclude that amifostine alone or in combination with EPO has limited therapeutic activity in MDS.

Aged↗
PubMed2001

Hereditary red blood cell disorders in middle eastern patients.

Hereditary disorders of erythrocytes are common in many areas of the world, including the Middle East. In some regions of the Middle East more than 10% of the population are carriers of a gene for one of these conditions. When patients from the Middle East seek medical care in the West, an unrecognized but clinically important erythrocyte disorder can result in serious complications during routine medical care, such as a drug-induced hemolytic crisis. This article reviews the most important and most common inherited red blood cell disorders in Middle Eastern patients, including glucose-6-phosphate dehydrogenase deficiency, the thalassemias, and sickle cell disorders. We discuss when to suspect such conditions, how to determine their presence, and how to avoid potential complications related to them. Although a detailed discussion of treatment of erythrocyte disorders is beyond the scope of this article, some general management principles are described.

Anemia, Hemolytic, Congenital↗
PubMed2001

"Congo" red: out of Africa?

CONTEXT: Congo red is the essential histologic stain for demonstrating the presence of amyloidosis in fixed tissues. To the best of my knowledge, nothing has been written about why the stain is named "Congo." OBJECTIVE: To understand the etymology and history of the Congo red histologic stain. DESIGN: Primary sources were consulted extensively, including 19th-century corporate documents, newspapers, legal briefs, patents, memoirs, and scientific papers. SETTING: Sources were obtained from multiple university libraries and German corporate archives. RESULTS: To Europeans in 1885, the word Congo evoked exotic images of far-off central Africa known as The Dark Continent. The African Congo was also a political flashpoint during the Age of Colonialism. "Congo" red was introduced in Berlin in 1885 as the first of the economically lucrative direct textile dyes. A patent on Congo red was filed by the AGFA Corporation of Berlin 3 weeks after the conclusion of the well-publicized Berlin West Africa Conference. During these important diplomatic talks, German Chancellor Otto von Bismarck presided over a discussion of free trade issues in the Congo River basin. A challenge to AGFA's Congo red patent led to a precedent-setting decision in intellectual property law. CONCLUSIONS: The Congo red stain was named "Congo" for marketing purposes by a German textile dyestuff company in 1885, reflecting geopolitical current events of that time.

Africa↗
PubMed2000

Littoral cell angioma associated with portal hypertension and resected colon cancer.

Littoral cell angioma (LCA) is a rare vascular tumor of the spleen with an unknown etiology and unclear natural history. An association with synchronous malignancy has been described. We report the case of a 54-year-old woman who had progressive splenomegaly over 3 years following resection of a colon adenocarcinoma. The splenomegaly was associated with portal hypertension and severe thrombocytopenia. Splenectomy was performed, and the histologic and immunocytochemical features of the spleen specimen were consistent with LCA. The relationship between LCA and malignancy is reviewed.

Adenocarcinoma↗
PubMed2000

Lymphadenopathy.

Lymphadenopathy can occur in any age group, in symptomatic or asymptomatic patients, and in a single site or at multiple sites. Lymphadenopathy is associated with numerous disorders. An abnormal lymph node may be observed or palpated by the patient, found by a health care worker, or discovered through radiologic evaluation. Lymphadenopathy may be a part of a complex case presentation, or the clinical cause may be straightforward. Patients with potentially curable malignant disorders may have lymphadenopathy as the first sign of their disease. This review of lymphadenopathy summarizes general considerations, discusses which patients might be considered for biopsy, reviews which nodes are most likely to be diagnostic, outlines initial diagnostic considerations on a region-by-region basis, and reviews a broad differential diagnosis for adenopathy.

Age Factors↗
PubMed2000

Autoimmune hemolytic anemia in a patient with autosomal dominant chronic mucocutaneous candidiasis.

Chronic mucocutaneous candidiasis is a heterogeneous immunodeficiency syndrome characterized by recurrent candidal infections of the skin, nails, and mucous membranes. The syndrome can be associated with autoimmune conditions, especially endocrine disorders. Typically, inheritance is autosomal recessive, and abnormal T-cell-mediated immunity is thought to be the underlying deficit. We describe a 27-year-old man with chronic mucocutaneous candidiasis inherited in an autosomal dominant fashion, in whom both lymphocyte blastogenesis and delayed-type skin reactivity to Candida antigens were normal. Notable features of the case include autoimmune hemolytic anemia, probable hypoparathyroidism, and hypogonadal hypogonadism.

Adult↗
PubMedDate not supplied

Myelodysplastic syndrome and pregnancy: the Mayo Clinic experience.

Although the myelodysplastic syndrome (MDS) is most common in the elderly, younger patients, including women of child-bearing age, may be affected. The association of MDS with pregnancy appears to be very rare: fewer than 25 cases have been reported. We report the outcomes of seven pregnancies in four women seen at the Mayo clinic between 1983 and 2000. Three of the women were found to have MDS when an abnormal complete blood count was detected during routine prenatal care. The fourth patient had an apparently congenital MDS, and suffered three spontaneous abortions before undergoing premature menopause as a result of pelvic irradiation for vulvar cancer. We discuss some of the unique concerns regarding pregnant patients with MDS. As women give birth at older ages and as more young persons survive cancer, MDS and pregnancy are likely to be seen together with increasing frequency.

Adult↗
PubMedDate not supplied

Hydroxyurea-associated platelet count oscillations in polycythemia vera: a report of four new cases and a review.

Cyclic oscillations in the peripheral blood platelet count were recently described in two patients with polycythemia vera (PV) receiving therapy with hydroxyurea (HU). This phenomenon can make proper HU dosing very challenging and may be especially problematic in PV patients who are at risk for thrombohemorrhagic complications. In this report, we describe four new cases of HU-associated platelet oscillations in patients with PV and extend our observations on one of the two previously reported cases. We also review cyclic thrombocytosis within the broader context of periodic hematopoiesis. A thrombopoietin-mediated negative feedback loop is central to most models of periodic thrombopoiesis, but this is probably an oversimplification. It is possible that HU destabilizes the delicate balance of thrombopoietin/c-Mpl signaling in megakaryocytic lineage hematopoiesis that is already markedly altered in PV; this hypothesis remains speculative. For patients who develop oscillatory variation in their platelet counts associated with HU use, keeping the HU dose constant may result in damping or termination of the cycles. However, this strategy is not always successful.

Adult↗