Clinical problem-solving. Costly errors.
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Aged↗
Biomedical subjects
T P Duffy
Publications and source records attributed to T P Duffy.
The mast cell and mast cell disease.
Mast cell disease or mastocytosis is a heterogeneous group of clinical disorders characterized by the proliferation and accumulation of mast cells in a variety of tissues, most often the skin. The signs and symptoms of mast cell disease are varied, dependent on the localization of mast cells in different organs and the local and systemic effects of mediators released from these cells. Although mast cell disease is most commonly identified in the skin, involvement of the skeletal, hematopoietic, gastrointestinal, cardiopulmonary, and central nervous systems may be seen. Clinical management of mastocytosis depends most heavily on knowledge of the diverse effects of mast cell mediators on various tissues and organs, the stimuli that can cause their release, and the different methods available for blocking the effects of these mediators.
Bone Diseases↗
Anemia of pregnancy.
A general work-up for anemia during pregnancy could follow a simplified flow diagram, such as the one shown in Figure 1. More specific laboratory tests should be selected as needed.
Anemia↗
Clinical problem-solving. An anatomy lesson.
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Granuloma↗
Clinical problem-solving. Remembering the ABC's.
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Adult↗
Pericardial tamponade in chronic myelomonocytic leukemia.
We report four cases of malignant pleuropericardial effusions in patients with chronic phase myelomonocytic leukemia (CMML). Based on current literature, this event is rare and very poorly understood. Our cases shed light on three important clinical characteristics: (1) patients with CMML develop effusions during uncontrolled leukocytosis; (2) these effusions are very responsive to conventional chemotherapy; and (3) effusions may develop without clinical forms of disease in other sites of extramedullary hematopoiesis.
Aged↗
Clinical problem-solving. The sooner the better.
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Adult↗
Sickle myonecrosis revisited.
This review focuses on the classification of sickle myonecrosis in the context of sickle cell vaso-occlusive crisis. Further, the potential and novel use of magnetic resonance imaging to further clarify the nature of sickle crisis is discussed.
Adult↗
Isolated amyloidosis of the penile urethra and corpus spongiosum: a case report.
Localized amyloidosis of the penile urethra and corpus spongiosum is rare. The pathogenesis is obscure and optimal management of such patients has not been defined. We report a case that was treated with transurethral removal of amyloid tissue, which was obstructing the urethral outlet. Previously, the patient had been treated with dilation with temporary relief of the symptoms. He was disease-free 1 1/2 years postoperatively.
Adult↗
Successful childbirth by a patient with congenital factor XI deficiency and an acquired inhibitor.
Acquired inhibitors in factor XI deficiency (FXI) are rare. The presence of an inhibitor during pregnancy poses a potential haemorrhagic risk to the fetus. We report an uncomplicated pregnancy and successful childbirth by a woman with congenital FXI deficiency and an acquired inhibitor, and discuss the persistence of residual FXI activity in the presence of an inhibitor.
Adult↗
Disseminated intravascular coagulation and excessive fibrinolysis in a patient with metastatic prostate cancer. Response to epsilon-aminocaproic acid.
BACKGROUND: Disseminated intravascular coagulation (DIC) and primary fibrinolysis have both been reported in association with prostate carcinoma. The correct diagnosis of the coagulopathy can be difficult and the appropriate management controversial. METHODS: A case is presented of a man in whom DIC and soft tissue hemorrhage developed after prostatic biopsy. The results of therapy and a review of the literature are discussed. RESULTS: Fibrinogen levels continued to decrease despite high-dose estrogen therapy, but they rapidly returned to normal after therapy with epsilon-aminocaproic acid. Although routine coagulation tests were suggestive of primary fibrinolysis, the results of the D-dimer assay confirmed that the patient had DIC associated with excessive fibrinolysis. CONCLUSION: A review of the literature suggests that most cases of primary fibrinolysis are probably DIC with excessive secondary fibrinolysis. In cases in which bleeding is the primary manifestation of DIC and there is a significant reduction in alpha-2-plasmin inhibitor activity, a trial of epsilon-aminocaproic acid and low-dose heparin should be considered. The failure in this case of estrogen therapy to correct the coagulopathy, despite a later good tumor response, is consistent with the delay in which anorchid testosterone levels are obtained after initiating treatment.
Adenocarcinoma↗
The many pitfalls in the diagnosis of myeloma.
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Aged↗
Morphologic, biochemical, and cytogenetic studies of bone marrow and circulating blood cells in painters exposed to ethylene glycol ethers.
In a previous cross-sectional survey, up to 15% of shipyard painters were found to have mild anemia or granulocytopenia, mostly acquired since employment. Environmental studies had suggested a possible etiologic role for ethylene glycol ethers, solvents to which the men were heavily exposed and which have established myelotoxic potential. To exclude alternative hypotheses, examine possible common patterns of injury, and identify potential risk factors and markers for such an effect, the affected painters were further studied. The painters were matched with two groups of controls: exposed painters without evidence of hematologic abnormality on the previous survey and unexposed controls. Altogether 25 subjects were studied by histopathologic examination of bone marrow, cytogenetic studies of marrow cells, and peripheral lymphocytes and peripheral red cell studies of membrane and metabolic function. Except for an unexpected finding of a race-associated effect on marrow histology, insignificant differences were seen among the groups in terms of marrow morphology and cellularity, stem cell growth kinetics, and marrow or peripheral cytogenetics. Two metabolic abnormalities of peripheral red cells related to exposure or clinical status of the subjects were found. Pyruvate kinase, an established marker of acquired myelodysplasia, was significantly depressed in the subjects with previously abnormal counts. Although reduced glutathione levels and holoenzyme activities of glutathione reductase (GSHR) did not differ among groups, exposed subjects had decreased saturation of GSHR with flavin adenine dinucleotide which could be restored in vitro, suggesting riboflavin deficiency or impaired riboflavin metabolism. Thus, although a unique pattern of bone marrow injury by histologic or genetic assay attributable to ethylene glycol ethers was not defined, biochemical effects of possible mechanistic importance were identified. The relevance of these findings as subclinical disease markers remains to be established.
Adult↗
Interferon therapy for agnogenic myeloid metaplasia complicated by immune hemolytic anemia.
A 69-year-old man with agnogenic myeloid metaplasia was treated with interferon-alpha 2 as part of a Phase II clinical trial. The patient responded to this treatment with a definite improvement in bone marrow histology, demonstrating increased numbers of hematopoietic colonies and partial resorption of the myelofibrosis. Chromosomal analysis on 20 cells suggested the re-emergence of normal hematopoietic progenitor cells: whereas previously, all metaphase spreads demonstrated a deletion in chromosome 20, the patient was now chimeric, with two of 20 cells exhibiting a normal karyotype. Nevertheless, the patient's anemia progressed during interferon therapy, with the development of an immunologically mediated hemolytic disorder. This hemolytic process developed after prolonged treatment with interferon, accelerated during therapy, and resolved following splenectomy and withdrawal of the drug. Initially, screening tests failed to detect the presence of the autoantibody. Similar immunologic processes may have been overlooked in other patients treated with interferon, especially if tests for autoantibodies were obtained early in their course of treatment. This case suggests a therapeutic role for interferon-alpha 2 in the management of the myeloproliferative diseases. It is presented, too, to underscore the immunomodulatory potential of the biologic response modifiers and their capacity to induce immunologic disorders.
Aged↗