Hb H disease in association with the silent beta chain variant Hb Hamilton or alpha 2 beta 2(11)(A8)Val----Ile.
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Biomedical subjects
Publications and source records attributed to R Liang.
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Seventeen nodal lymphomas (originally diagnosed as T-cell lymphomas based on histological features and immunohistochemical staining results) were studied for the presence of Epstein-Barr virus (EBV) genome, and the results correlated with immunoglobulin and T-cell receptor gene rearrangement analyses performed on the same tissue samples. All four EBV positive cases had clonal rearrangement of the joining region of the immunoglobulin heavy chain (IgJH) gene without clonal T-cell receptor beta-chain (TCR beta) gene rearrangement. Of these, two cases also showed clonally rearranged light chain gene, and they were reclassified as T-cell rich B-cell lymphomas (TRBL). The other two cases lacked clonal kappa or lambda light chain rearrangement and they were reclassified as T-cell rich lymphomas of probable B lineage, based on their isolated IgJH clonal rearrangement. These B-cell lymphomas may be easily misdiagnosed as T-cell lymphomas owing to the presence of an abundant reactive T-cell infiltrate masking the tumor population. The florid T-cell reaction may represent an unusual host response towards a clonal proliferation of EBV bearing B cells.
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Polymerase chain reaction (PCR) was used to amplify the DNA fragments of the complementarity determining region 3 of the immunoglobulin (Ig) gene heavy chain from the leukemic cell specimens of patients with acute and chronic lymphoid leukemias of B-cell lineage. Two different pairs of primers were tested. Fourteen of the 17 (82%) cases of acute lymphoblastic leukemia (ALL), and all 15 cases (100%) of B-cell chronic lymphocytic leukemia, who had rearrangement of the Ig gene heavy chain by Southern analysis, were positive by PCR with either one or both pairs of primers. This technique was able to detect leukemic cells at the level of 0.1%. Applying it to study the remission marrow specimens following induction chemotherapy was more useful than morphology alone in predicting early relapse of the leukemia.
A total of 29 evaluable patients with acute myeloid leukaemia (AML) either in relapse or resistant to initial induction daunorubicin-containing chemotherapy were given a salvage regimen consisting of moderate-dose cytosine arabinoside and mitoxantrone. There were 8 (28%) complete responders (CRs), 4 (14%) partial responders (PRs), and 17 (52%) nonresponders. The duration of CRs was 2+, 2+, 3+, 3, 4+, 4, 5 and 6 months respectively. Two of the eight CR patients were refractory to initial daunorubicin-containing induction therapy and another two had achieved a previous CR lasting less than 6 months. Four of the eight CR patients had received an amsacrine-containing salvage regimen (ATA) prior to administration of the present moderate-dose cytosine arabinoside and mitoxantrone regimen; this indicates the lack of absolute clinical cross-resistance between the present combination and the daunorubicin- or amsacrine-containing regimens. However, the duration of CRs achieved by these patients remains very short and should, if possible, be followed by allogeneic or autologous bone marrow transplantation.
A total of 20 adults patients presenting with previously untreated lymphoblastic lymphoma underwent an intensive chemotherapy protocol. Either the BACOP or the m-BACOD regimen was used for induction. If the patients achieved a complete clinical remission (CR) after three courses, they were given intensive consolidation and maintenance chemotherapy based on a protocol that was modified from the L10/L17M regimen of the Memorial Sloan-Kettering group for acute lymphoblastic leukaemia and lymphoblastic lymphoma. Patients exhibiting localised areas of bulky disease were given additional involved-field radiotherapy. In all, 15 (75%) men and 5 (25%) women were entered in this study. Their median age was 28 years (mean, 30 years; range, 12-64 years). Overall, 3 (15%) had stage II disease, 3 (15%) had stage III disease and 14 (70%) had stage IV disease; 7 (35%) patients exhibited B symptoms and 4 (20%) had bulky disease. The overall (CR) rate was 10/20 (20%), and that following BACOP and m-BACOD therapy was 4/8 (50%) and 6/12 (50%), respectively. In all, 7 of the 10 complete responders (70%) relapsed. The disease-free survival of the ten who achieved a CR was 23% at 3 years. The overall survival of all 20 patients at 3 years was only 37%, and there were very few long-term survivors. More effective treatment for adult lymphoblastic lymphoma is required.
A total of 176 patients with gastrointestinal lymphomas were reviewed. According to a modified staging classification, 51 of them had stage I/II disease and the remaining 125 had stage III/IV disease. In most cases (68%), the histology was intermediate-grade according to the NIH working formulation, and the B-cell immunophenotype was involved in 89% of the 45 cases with a known immunophenotype. The primary site was the stomach in 56% of cases and the bowel in 44%. A significantly higher proportion (P = 0.001) of those with bowel lymphomas had stage III/IV disease (88% vs 57%). The primary gastrointestinal lesion was resected in 122 patients, including all 51 cases of stage I/II disease. In all, 8 stage I/II patients were given radiotherapy alone following surgery and the other 43 underwent chemotherapy; of the latter, 19 received additional radiotherapy following chemotherapy. Chemotherapy was also given to 112 stage III/IV patients, 42 of whom underwent additional radiotherapy. Factors associated with a poorer prognosis included advanced disease, bowel lymphoma and advanced age. Although the complete response (CR) rate according to disease stage was similar, stage I/II patients receiving chemotherapy showed a significantly lower relapse rate, better disease-free survival following CR and improved survival as compared with those receiving radiotherapy alone. However, additional radiotherapy following chemotherapy did not further improve the clinical outcome.
A total of 92 patients with previously untreated intermediate- or high-grade non-Hodgkin's lymphoma attending the University Department of Medicine, Queen Mary Hospital, Hong Kong, were treated with the m-BACOD chemotherapy regimen (methotrexate, bleomycin, doxorubicin, cyclophosphamide, vincristine and dexamethasone). Additional involved-field radiotherapy was given to 32 (35%) patients. Myelosuppression was the major toxicity, and 5 (5%) treatment-related deaths occurred due to pneumonia, bleomycin sensitivity, doxorubicin cardiotoxicity and reactivation of hepatitis B infection. The overall complete response (CR) rate was 65/92 (71%) and the relapse rate was 22/65 (34%). The disease-free survival of the 65 CR patients at 2 years was 52% and the overall survival of all 92 patients at 3 years was 56%. The CR rate of stage I and II patients was significantly better than that of those with stage III and IV disease (87% vs 59%; P = 0.01), and the CR rate of stage III patients was superior to that of those with stage IV disease (86% vs 50%; P = 0.05). The overall survival of stage III and IV patients was significantly worse than that of subjects with stage I and II disease (31% vs 73%; P = 0.02). Multivariate analysis revealed that the independent prognostic variables significantly determining the CR rate and survival included the clinical stage and the serum lactate dehydrogenase level. From this study, the results of treatment with the m-BACOD regimen in patients with advance disease appeared to be similar to those obtained using the conventional CHOP regimen (cyclophosphamide, doxorubicin, vincristine and prednisone).
The clinical outcome of 149 patients with Stage I-II intermediate grade non-Hodgkin's lymphomas was analysed. There were 77 (52%) males and 72 (48%) females. Their median age was 58 years. Eighty patients (54%) had Stage I disease and 69 (46%) Stage II. Twenty-nine (20%) of them received radiotherapy only, 75 (50%) doxorubicin-containing chemotherapeutic regimens and 45 (30%) other less intensive chemotherapy. Sixty-two patients had additional radiotherapy following chemotherapy. Patients receiving other less intensive chemotherapy were significantly older (P = 0.0001) and those receiving radiotherapy alone had a significantly higher proportion of Stage I disease (P = 0.007). Patients receiving less intensive chemotherapy had a significantly low complete response (CR) rate. Patients receiving radiotherapy alone had a significantly high relapse rate and low disease-free survival following CR. However, there was no significant difference in overall survival regardless of the mode of therapy. This lack of survival advantage might be related to the effective salvage chemotherapy in some of the radiotherapy failures and the occasional chemotherapy-related mortality. Additional radiotherapy following chemotherapy did not appear to give further benefit.
Ten patients with lymphomas involving the parotid gland were studied. There were five males and five females. Their median age was 64 years. There were three cases of stage I disease, two stage II and five stage IV. The histology was small lymphocytic type in four of the five stage IV patients and two of them were associated with Sjögren's syndrome. The other stage IV patient had a diffuse small non-cleaved cell lymphoma. A different pattern of the histology was observed in the five stage I/II patients. They all had an intermediate grade histology and were not associated with Sjögren's syndrome. It was possible that at least some of the patients in the latter group might have their lymphomas arising from the lymph nodes within the gland rather than in the salivary gland parenchyma.
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The pattern of immunoglobulin (Ig) and T cell receptor (TCR) gene rearrangements was determined in 87 patients with acute and chronic leukaemias and myelodysplastic syndromes by Southern blot hybridisation. All 31 cases of common, B cell and null cell acute lymphoblastic leukaemia, and B cell chronic lymphocytic leukaemia showed Ig heavy chain (JH) rearrangement, and TCR (beta-chain) rearrangement was seen in all 5 cases of T cell acute lymphoblastic leukaemia. Inappropriate JH and TCR (beta) rearrangements were present in some cases of T-ALL (60%) and common acute lymphoblastic leukaemia (18%), respectively. For the 19 patients with acute leukaemias following chronic myeloid leukaemia, blastic transformation, all 4 with lymphoid transformation and 3 of the 15 with myeloid transformation had JH rearrangement, and 3 CD10-positive lymphoid transformation and 2 myeloid transformation had their TCR (beta) genes rearranged. In conclusion, the pattern of Ig and TCR gene rearrangements correlated well with the cell lineage. However, cross-lineage rearrangements were more commonly seen in patients with acute leukaemias following chronic myeloid leukaemia blastic transformation, as compared to the de novo cases.
The aim of this study was to determine the effect of immunophenotype on the clinical characteristics and prognosis of 144 adult Chinese patients with non-Hodgkin's lymphomas. Those entities with well-recognised immunophenotype and clinical characteristics were excluded. Significantly more patients with T-cell lymphomas had B symptoms (52 vs. 30%, p = 0.05) and fewer had bulky disease (7 vs. 25%, p = 0.04). Extranodal involvements of liver, spleen, marrow, nasal region and skin were significantly more common in T-cell lymphomas. On the other hand, gastro-intestinal involvement was more commonly seen in B-cell tumours. Induction chemotherapy of comparable intensity was used in treating these patients. The immunophenotype did not appear to affect significantly their prognosis.
A case of acquired haemophilia A presenting with extensive spontaneous bruising and anaemia is reported. The anaemia was due to myelodysplastic syndrome (FAB: refractory anaemia with ringed sideroblasts). A factor-VII:C-specific inhibitor was also found. Prednisone and pyridoxine were given, and the inhibitor became undetectable after 4 weeks of therapy, but the abnormal ringed sideroblasts still persisted on repeated bone marrow biopsy.
During a 14-year period, 122 of 840 (14.5) Hong Kong patients with non-Hodgkin's lymphomas were diagnosed to have low-grade lymphomas according to the Working Formulation. Only 28 (23%) of them had stage I and II disease and local radiotherapy appeared to be curative in some of them. Although remissions were achieved with chemotherapy in a majority of the stage III and IV patients, a pattern of continuous relapses was noted in their disease-free survival curve. Multivariate analysis revealed that clinical stage and presence of B symptoms were significant independent prognostic factors.
The management of 47 patients with stage I and II Waldeyer's ring lymphomas was reviewed. Twenty-one of them received radiotherapy only, and the other 26 received combined chemotherapy and radiotherapy. Patients with primary disease in the nasopharynx, high-grade histology or advanced age were associated with poorer survival. Although the complete response rates were similar, the patients receiving combined chemotherapy and radiotherapy had significantly fewer relapses and a better disease-free survival. However, their survivals were similar. The lack of improvement in the overall survival might be due to the effectiveness of salvage chemotherapy in treating some of the radiotherapy failures and the occasional chemotherapy-related mortality.
A 38-year-old woman with acute myeloid leukaemia developed focal hepatic candidiasis. Ultrasonography and computerised tomography revealed the hepatic lesion and definite diagnosis was established by percutaneous liver biopsy. The use of a cumulative dose of 6.6 g of amphotericin B in combination with 5-fluorocytosine resulted in successful eradication of the fungus. The difficulties in making an antemortem diagnosis are well recognised and optimal therapy of the condition remains to be defined.