We report strongyloides hyperinfection in two patients with generalized hypogammaglobulinemia from multiple myeloma and nephrotic syndrome, despite a significant strongyloides-specific immunoglobulin G (IgG) response. In contrast to reports on animals, where human IgG was shown to be a protective antibody, our observation suggests that in humans, immunity to the infective-stage larvae is not protective against the autoinfective larvae, which are the causative agents of strongyloides hyperinfection. Case reports. (i) Patient 1.A 55 year-old Chinese male with immunoglobulin A() [IgA()] multiple myeloma developed constipation and nonspecific abdominal pain after receiving vincristine, adriamycin, and dexamethasone as part of his chemotherapeutic regimen. The clinical examination was initially unremarkable, and both chest and abdominal radiographs were normal. A complete blood count revealed leukocytosis, total white cells, 12.1 ϫ 10 9 /liter (eosinophils, 0.3%), with normal hemoglobin (14.3 g/dl) and platelet (220 ϫ 10 9 /liter) levels. Electrolyte assay was significant for hyponatremia (sodium, 119 mmol/dl) and was otherwise unremarkable (potassium, 4.7 mmol/dl; blood urea, 4.9 mmol/liter; creatinine, 32 mol/dl; glucose, 7.2 mmol/liter). Liver function test was normal, apart from albumin of 26 g/liter and globulin of 20 g/liter. Serum and urine osmolality were consistent with the syndrome of inappropriate antidiuretic hormone production. His initial symptoms had responded partially to laxatives.Two days later, he became hypotensive and a repeat complete blood count showed a fall in the hemoglobin level to 9.1 g/dl. Emergency gastroscopy revealed an area of erythematous and granular mucosa in the second part of the duodenum resembling lymphangiectasia with no evidence of bleeding. This was biopsied. His conditions stabilized after fluid resuscitation. However, he became acutely dyspneic and hypoxemic the following day. A repeat chest radiograph showed increased pulmonary infiltrates bilaterally. He was ventilated and managed in the intensive care unit. Bronchoalveolar lavage was performed.The duodenal biopsy revealed multiple filariform larvae of Strongyloides stercoralis within the glandular crypts, with marked surrounding inflammatory infiltrates. Microscopic examination of the smears obtained by bronchoalveolar lavage also showed a large number of larvae. The detection of larvae in both the duodenum and by bronchoalveolar lavage confirmed strongyloides hyperinfection in this patient. Strongyloides-specific IgG antibody (by an enzyme-linked immunosorbent assay method against the soluble antigen derived from processed S. stercoralis larvae L3) was elevated at 4.74 (normal, Ͻ1.00) (IVD Research, Carlsbad, CA). Tests for other strongyloides-specific immunoglobulin subclasses were not performed. Human immunodeficiency virus and human T-lymphotropic virus type 1 tests were negative. Oral ivermectin (200 g/kg) was prescribed for 10 days with good clinical and radiological recovery. He was discharged 12 days later, and ...
A 60-year-old woman, with no significant past medical history, presented with syncope secondary to complete heart block. Emergency cardiac pacing was performed with clinical improvement. The initial complete blood count was normal: WBCs, 12.03 ¥ 10 9 per L; Hb level, 13.8 g per dL; and PLTs, 250 ¥ 10 9 per L. Two days later she became hypotensive. An urgent 2-D echocardiogram revealed a moderate-sized pericardial effusion. A complete blood count then showed a PLT count of 84 ¥ 10 9 per L.After transfusing 6 units of PLT concentrates, the PLT count increased to 142 ¥ 10 9 per L. A pericardiocentesis was performed with improvement of clinical status. Three days later, a repeat complete blood count revealed a PLT count of 8 ¥ 10 9 per L. The patient was supported with further PLT transfusions, and a marrow examination was planned. The case was also referred to the hematology team. A review of the peripheral blood films disclosed severe PLT clumping in both samples taken in EDTA (left panel) and citrate (right panel, both, original magnification ¥ 400) tubes. The marrow examination was cancelled and the patient was discharged well.Pseudothrombocytopenia is an uncommon in vitro phenomenon caused by antibody-mediated PLT agglutination, which can be EDTA-or citrate-dependent or, even rarer (<0.02% of all blood counts), both. It does not pose any hemorrhagic risk, and its only clinical importance resides in its lack of recognition, which potentially could lead to inappropriate PLT transfusion, additional testing, and delays in diagnostic or therapeutic procedures. To avoid these, it is imperative that a peripheral blood film be examined and a complete blood count repeated using a different anticoagulant in an asymptomatic patient who gives unexpected low PLT counts, as illustrated in this case.
Biphenotypic acute leukemias (BALs) are uncommon. Most are of myeloid-B-cell or myeloid-T-cell lineage. We report herein a 70-year-old man with an unusual acute leukemia where the blasts expressed both B-and T-lymphoid markers. He presented to us with an enlarging cutaneous tumor. The presenting peripheral blood and bone marrow aspirate showed 40% and 90% blasts, respectively, which were negative for the usual cytochemical stains. The flow cytometric analysis revealed that the blasts were positive for CD19, CD20, CD22, cytoplasmic (Cyt) CD79a, CD10, Cyt CD3, CD5, CD7, CD4, HLA-DR, TdT, and were negative for myeloid markers. According to the scoring system from the European Group for the Immunological Characterization of Acute Leukaemias (EGIL), this case was an unequivocal B-cell/T-cell BAL. Conventional cytogenetic analysis revealed 46XY [t(4;11)(q31;q13), add(8)(q24), der(9)del(9)(p21)del(9)(q32q34), -13, +mar] in all 25 metaphases analyzed. Fluorescence in situ hybridization (FISH) and polymerase chain reaction (PCR) for 11q23 rearrangements as well as t(9;22) were negative. PCR for both TCR-g and IgH gene analyses revealed polyclonal rearrangements. We postulate that this case of BAL might have arisen from the putative common lymphoid progenitor cell. Am.
Background: Multiple myeloma (MM) is an incurable malignancy. Recent insights into its biology has allowed the use of novel therapies targeting not only the deregulated intracellular signaling in MM cells but also its interaction with the bone marrow microenvironment that confers drug resistance, growth, and survival advantage to the malignant cells. Methods: We review and summarize the recent advances in our knowledge of myeloma biology as well as the mechanism of action and clinical efficacy for novel therapeutic agents in clinical trials. Results: Several novel therapeutic agents are currently in clinical trials. Thalidomide is already established for both initial and salvage treatment. Bortezomib is being tested alone and in combination with conventional chemotherapy in various settings. Other agents are less effective in producing response but have been able to stabilize disease in patients with relapsed and/or refractory disease, such as arsenic trioxide, farnesyltransferase inhibitors, 2-methoxyestradiol, and vascular endothelial growth factor receptor inhibitors. Insights into drug resistance mechanism have also led to the development of novel agents that sensitize myeloma cells to chemotherapy (Bcl-2 antisense). Gene expression studies have in many instances identified pathways other than the intended target of the drug and have provided insights into the therapeutic mechanisms. Conclusions: In the future, patients with MM will have more therapeutic options available than ever before. The challenge will be to identify patient subgroups that will benefit most from the different therapies and then determine how these biologically based therapies could be combined and incorporated into the overall management of patients.
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