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C C So

Publications and source records attributed to C C So.

At least 19 recordsLinked to original sources

Cytomegalovirus infection associated with clonal proliferation of T-cell large granular lymphocytes: causal or casual?

Clonal proliferation of T-cell large granular lymphocytes (LGL) is an indolent disorder characterized by splenomegaly, lymphocytosis and frequent manifestations of immune disturbances. The LGL are CD3(+) CD4(-) CD8(+) CD56(-). The clonality of the tumor cell population is often only demonstrable by T-cell receptor (TCR) gene rearrangement study because chromosomal abnormality is distinctly rare. We describe a case of T-cell LGL leukemia that presented initially as cytomegalovirus infection. The leukemic LGL are shown to be clonal by both TCR gene rearrangement and chromosomal studies. They persist after subsidence of the cytomegalovirus infection.

Adult↗

Consolidation therapy with autologous blood stem cell transplantation in a patient with primary plasma cell leukaemia.

Primary plasma cell leukaemia (PPCL) is a rare form of plasma cell dyscrasia. Conventional melphalan-based treatment is often ineffective, with a reported median survival of 2-7 months only. We report a 53-year-old man with PPCL who was treated with four cycles of combination chemotherapy including vincristine, adriamycin and dexamethasone that resulted in a good partial remission. High-dose melphalan 200 mg/m2 and autologous peripheral blood stem cell (PBSC) rescue was then given 6 months after diagnosis. Maintenance interferon-alpha was started 8 weeks after transplantation with good drug compliance. Complete remission was achieved and molecular remission was documented 11 months after autologous PBSC transplantation. In conclusion, high-dose therapy followed by autologous stem cell rescue is a feasible option for PPCL that can result in a reasonably sustained remission.

Antineoplastic Combined Chemotherapy Protocols↗

Arsenic trioxide in comparison with chemotherapy and bone marrow transplantation for the treatment of relapsed acute promyelocytic leukaemia.

BACKGROUND: The best overall treatment strategy for patients with acute promyelocytic leukaemia (APL) in relapse with chemotherapy, bone marrow transplantation (BMT) or arsenic trioxide (As(2)O(3)) based therapy remains undefined. PATIENTS AND METHODS: We reviewed the clinical course and treatment outcome of 143 APL cases seen in four major hospitals in Hong Kong over a 10-year period. RESULTS: Complete remission (CR) was attained in 113 cases (79%) with all-trans retinoic acid (ATRA) and chemotherapy. Relapse occurred at a median of 16 months in 54 cases, with a 3-year disease free survival of 56%. Post-relapse treatment was successful in 41 cases (76%), giving an actuarial 3-year overall survival (OS) of 81% from CR1. Three different protocols were used: chemotherapy alone (n = 19), allogeneic BMT (n = 14) and an As(2)O(3)-based regimen (n = 21). Chemotherapy was associated with the highest treatment-related mortality (TRM) at 53%, giving a CR2 rate of 47%. TRM was 36% for BMT. The CR2 rate for the As(2)O(3)-based regimen was 100%, with no TRM. However, 38% of As(2)O(3) treated patients had subsequent relapses, which were further salvaged in 75% by combined As(2)O(3) plus ATRA. The actuarial OS for the three protocols leveled off by 2 years at 82% for As(2)O(3), 43% for BMT and 23% for chemotherapy (P = 0.0004). CONCLUSIONS: Our results suggest that As(2)O(3) may be superior to chemotherapy and BMT for the treatment of APL in relapse.

Adolescent↗

A novel t(2;3)(q31;p13) in acute myelocytic leukemia.

We report a case of acute myelocytic leukemia without maturation exhibiting a novel t(2;3)(q31;p13). Conventional cytogenetics showed the concomitant occurrence of a single metaphase with 47,XX,+8. Nevertheless, interphase cytogenetics by fluorescence in situ hybridization using a chromosome 8 alpha-satellite DNA probe showed that the percentage of cells with three hybridization signals was within the control range.

Chromosomes, Human, Pair 2↗

Hypoplastic myelodysplastic syndrome-a clinical, morphologic, or genetic diagnosis?

We report a middle-aged female with an 11-year history of nonprogressive pancytopenia and severely hypoplastic marrow with minimal morphologic dysplasia. A diagnosis of hypoplastic myelodysplastic syndrome (MDS) was made because of the finding of a persistent clonal abnormality, del(13)(q12q14), and the subsequent demonstration of a single Auer rod-containing blast in the peripheral blood smear. The case illustrates the problems in the differentiation between aplastic anemia and hypoplastic MDS.

Adult↗

Precursor T-lymphoblastic leukemia with a novel t(1;22)(p34;q13).

A 37-year-old woman that presented with cervical lymphadenopathy and leukocytosis was found to have precursor T-lymphoblastic leukemia (T-ALL). Cytogenetic study of the leukemic cells showed a 46,XX, t(1;22)(p34;q13) karyotype. The t(1;22)(p34;q13) is a novel chromosomal abnormality in human malignancies and is probably a variant form of the t(1;14)(p34;q11) found in precursor T-ALL.

Adult↗

Gain of chromosome 3/3q in B-cell chronic lymphoproliferative disorder is associated with plasmacytoid differentiation with or without IgM overproduction.

Trisomy 3 has been reported to be associated with marginal zone B-cell lymphoma. However, its occurrence and significance in other B-cell chronic lymphoproliferative disorders has not been fully defined. We report five cases of B-cell chronic lymphoproliferative disorders showing gain of chromosome 3 or 3q. The patients were elderly males who presented with splenomegaly with or without hepatomegaly and lymphadenopathy. The diagnoses included chronic lymphocytic leukemia (3 cases), prolymphocytic leukemia (1 case), and Waldenstrom macroglobulinemia (1 case). Distinctive feature in this group of patients was the plasmacytoid appearance of the leukemic lymphocytes, with an associated IgM hypergammaglobulinemia in three patients. The relationship between the gain of chromosome 3 and plasmacytoid differentiation in B-cell chronic lymphoproliferative disorders is discussed.

Aged↗

Chronic lymphocytic leukemia with a novel der(8;15)(q10;q10) translocation.

A 71-year-old male was found to have chronic lymphocytic leukemia. Cytogenetic study of the leukemic lymphocytes shows a 46,XY,der(8;15)+15. The der(8;15) is a novel chromosomal abnormality in human malignancies. The resulting loss of 8p and trisomy 15q are both unusual chromosomal changes in chronic lymphocytic leukemia.

Aged↗

Idiopathic myelofibrosis with extramedullary haemopoiesis involving the urinary bladder in a Chinese lady.

Extramedullary haemopoiesis (EMH) associated with idiopathic myelofibrosis most commonly involves the reticuloendothelial organs, such as the spleen and liver, although ectopic haemopoietic tissue has also been described rarely in the lymph nodes, skin, gastrointestinal tract, pleura, peritoneum, central nervous system, and genital and urinary tracts. We report on a 54-year-old Chinese lady with a long history of idiopathic myelofibrosis who presented with gross haematuria and left hydronephrosis due to EMH in the bladder trigone. Cystoscopic examination revealed a sessile necrotic papillary growth at the trigone, obstructing the left ureteric orifice. Transurethral resection of the bladder tumour was performed, and microscopic examination of the tumour chips demonstrated atypical megakaryocytes, immature granulocytes and normoblasts, confirming the presence of EMH. The residual bladder tumour responded well to low dose radiotherapy, with subsequent disappearance of haematuria and normalization of ultrasonogram findings.

Cell Lineage↗

Waldenström macroglobulinemia with karyotypic aberrations involving both homologous 6q.

An 84-year-old female presenting with proptosis and hyperviscosity syndrome was found to have Waldenström macroglobulinemia. Karyotypic analysis showed structural chromosomal abnormalities involving both homologous chromosomes 6 with a deleted 6q at q21-q23 and a complex three-break rearrangement in the t(6;13;21)?(q21;q14;q11). A literature review suggests that deletions of chromosome 6 at 6q21 are associated with lymphoplasmacytoid differentiation and IgM production in B-cell chronic lymphoproliferative disorders.

Aged↗

Acute myeloid leukemia with concomitant trisomies 4 and 10: a distinctive form of myeloid leukemia?

The occurrence of trisomy 4 or trisomy 10 as the sole chromosomal abnormality in acute myeloid leukemia (AML) is very rare, the reported frequency being less than 1%. We describe two cases of AML-M2 with concomitant trisomy 4 and trisomy 10, a hitherto undescribed phenomenon. They showed two unusual features, including immunoreactivity for CD56 and a short-lived but rapidly progressive myelodysplastic phase preceding the appearance of frank leukemia. These findings raise the possibility that AML with concommitant trisomy 4 and trisomy 10 may constitute a distinctive subtype of AML.

Acute Disease↗

Sinonasal angiosarcoma with marrow involvement at presentation mimicking malignant lymphoma: cytogenetic analysis using multiple techniques.

Angiosarcoma of the head and neck most commonly involves the skin of the scalp or face; primary involvement of the sinonasal region is exceedingly rate. We report a patient with sinonasal angiosarcoma who showed marrow involvement at presentation. Marrow aspiration smears showed many large, often segregated blast-like cells, mimicking malignant lymphoma. However, trephine biopsy revealed formation of anastomosing vascular spaces by the tumor cells and immunoreactivity for CD31, supporting a diagnosis of angiosarcoma. DNA ploidy analysis showed an apparent diploidy. Nevertheless, conventional cytogenetics demonstrated very complex chromosomal abnormalities with the presence of multiple hypodiploid clones, together with several near-triploid to near-tetraploid clones showing structural abnormalities involving chromosomes 1, 3, 4, 9, 14, 16, 17, 18, and 22. The identification of these karyotypic changes has been facilitated by the application of comparative genomic hybridization and spectral karyotyping.

Adult↗

Deletion of Xq23 is a recurrent karyotypic abnormality in acute myeloid leukemia.

Deletion of chromosome Xq23 has been reported in a number of solid tumors, including soft tissue sarcoma, malignant melanoma, astrocytoma, and adenocarcinoma. The deleted Xq often occurs in a setting of very complex karyotypic changes. A similar abnormality has also been described in rare cases of acute myeloid leukemia (AML) but in no other hematologic malignancies. In this study, we report the occurrence of del(X)(q23) in two cases of AML.

Acute Disease↗

Cytogenetic triclonality in T-cell acute lymphoblastic leukemia: a conventional and molecular cytogenetic study.

Cytogenetically-unrelated clones are infrequently seen in hematologic malignancies, and are particularly uncommon in acute lymphoblastic leukemia. We report a case of T-cell acute lymphoblastic leukemia with L2 morphology which demonstrated three cytogenetically distinct clones: 46,XY,t(2;9)(p21;q34)/46,XY,del(6)(q21q23)/47,XX,+8. Interphase cytogenetic analysis by fluorescence in situ hybridization (FISH) confirmed the presence of trisomy 8 in a significant proportion of lymphoblasts, while reverse transcription-polymerase chain reaction (RT-PCR) did not show the presence of BCR/ABL fusion. This is the first report describing the occurrence of cytogenetic triclonality in de novo T-cell acute lymphoblastic leukemia.

Child↗

Alloimmunization in Chinese with warm autoimmune haemolytic anaemia - incidence and characteristics.

We perform a retrospective study to determine the incidence and characteristics of alloimmunization in Chinese patients with warm autoimmune haemolytic anaemia. Among 67 patients studied, clinically significant alloantibodies were found in only eight patients, giving a rate of alloimmunization of 11.3%. The latter contrasts with the much higher rate reported in the Western population. This probably reflects the genetic homogeneity in Chinese with respect to the red cell phenotype. The alloimmunization rate, however, is still significant and therefore comprehensive pretransfusion testing is required for Chinese patients with warm autoantibodies so as to safeguard patient's safety.

Adult↗