Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

One hundred seventy-four patients with progressive or advanced chronic lymphocytic leukemia (CLL) have received initial therapy with fludarabine as a single agent or fludarabine combined with prednisone. The overall response rate was 78% and the median survival was 63 months. No difference in response rate or survival was noted in the 71 patients receiving fludarabine as a single agent compared with the 103 patients who received prednisone in addition. The median time to progression of responders was 31 months and the overall median survival was 74 months. Patients over the age of 70 years had shorter survivals. Patients with advanced stage disease (Rai III and IV) had a somewhat shorter survival than earlier stage patients. More than half the patients who relapsed after fludarabine therapy responded to salvage treatment, usually with fludarabine-based regimens. Second remissions were more common in patients who had achieved a complete remission on their initial treatment. The CD4 and CD8 T-lymphocyte subpopulations decreased to levels in the range of 150 to 200/microL after the first 3 courses of treatment. Although recovery towards normal levels was slow, the incidence of infections was low in patients in remission (1 episode of infection for every 3.33 patient years at risk) and decreased with time off treatment. There was no association of infections or febrile episodes with the use of corticosteroids or the CD4 count at the end of treatment and a poor correlation with the increase in CD4 counts during remission. Infectious episodes were less common in patients who had a complete response compared with partial responders. Richter's transformation occurred in 9 patients and Hodgkin's disease occurred in 4 patients. Five other patients died from other second malignancies. Fludarabine appears to be an effective initial induction therapy with a reasonable safety profile for patients with CLL.
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PMID:Long-term follow-up of patients with chronic lymphocytic leukemia (CLL) receiving fludarabine regimens as initial therapy. 969 4

A case is presented of CD30+ anaplastic large cell lymphoma of the spleen. The patient, a 61 year old woman with a history of chronic lymphocytic leukemia (CLL) was seen for the sudden development of splenomegaly with thrombocytopenia. A splenectomy was performed which showed massive replacement of the spleen by a population of large atypical lymphoid cells showing bizarre nuclear forms and multinucleated tumor cells reminiscent of Reed-Sternberg cells. Immunohistochemical studies showed strong membrane and dot-like paranuclear positivity in the majority of the atypical cells for CD30, with coexpression in many of the cells for CD15. Additionally, the cells also strongly reacted with CD3, UCHL-1, EMA and LCA. The present case illustrates an unusual variant of anaplastic (CD30+) large cell lymphoma sharing histologic and immunophenotypic features that overlap with those of Hodgkin's disease. The history in this patient of CLL with sudden development of splenomegaly raises the possibility of transformation of CLL into a high-grade lymphoma (Richter's syndrome). The possible pathogenetic implications of this phenomenon are discussed.
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PMID:Anaplastic large cell lymphoma of the spleen. 972 68

Gallium (67Ga) scan was performed in 29 CLL patients with chronic lymphocytic leukemia who were suspected on clinical grounds to have Richter's transformation (RT). Of 29 patients, nine had a positive 67Ga scan; seven of these had a subsequent biopsy that verified large-cell lymphoma or Hodgkin's disease. The other two patients underwent biopsies that revealed fungal infections, a known cause of 67Ga uptake. Two patients had biopsies that were consistent with RT but showed no affinity to 67Ga. One false negative resulted five days after chemotherapy, a known cause of diminished 67Ga uptake. The other occurred within a small infraorbital mass, containing only 10% centroblasts, which is below the level of detection for 67Ga scanning. Subsequent 67Ga scans in both patients revealed 67Ga avid lesions, which demonstrated RT upon biopsy. This technique was more strongly predictive of RT than was measurement of serum B-2 microglobulin or serum lactate dehydrogenase levels. 67Ga scanning is very useful in localizing an optimal site for biopsy to document RT; it may also have the potential to help assess response to treatment, predict recurrence, and contribute to long-term follow-up in this subset of patients.
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PMID:The usefulness of high dose (7-10mci) gallium (67Ga) scanning to diagnose Richter's transformation. 1061 60

Chronic lymphocytic leukemia (CLL) is a chronic, low-grade hematologic malignancy that can transform to a large cell non-Hodgkin's lymphoma (Richter's syndrome), which is associated with an unfavorable prognosis. A distinct Hodgkin's disease subgroup of lymphomatous CLL transformation has been well characterized. We describe a patient presenting with simultaneous manifestations of CLL and Hodgkin's disease. This patient is unique, presenting with separate sites of involvement of each disease within the same organ, in this case the bone marrow. The morphologic and immunohistochemical findings at diagnosis are correlated with the findings of the postmortem examination.
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PMID:Simultaneous occurrence of Hodgkin's disease and chronic lymphocyte leukemia: a unique presentation. 1084 82

Approximately 3 to 5% of patients with chronic lymphocytic leukemia (CLL) develop an aggressive large cell non Hodgkin's lymphoma (NHL) known as Richter's syndrome (RS). RS has a poor prognosis and a response rate of < 10% with fludarabine-based or other cytotoxic combination regimens. The aim of this study was to evaluate the efficacy and toxicity of the hyperCVXD regimen in RS. Twenty-nine patients, median age 61 years (36-75) 23 males, were treated. Prior diagnosis was CLL in 26 patients, NHL in 2, and Prolymphocytic leukemia in 1. Treatment consisted of fractionated cyclophosphamide, vincristine, daunoXome and dexamethasone. Six patients (20%) died while receiving study therapy, 4 (14%) during the first cycle of whom 2 had started therapy with overt pneumonia. Grade 4 granulocytopenia occurred in all 95 cycles of therapy with a median time to recovery of 14 days. Twenty three (24%) cycles were complicated by fever, and 15 (15%) by pneumonia. Sepsis was documented in 8 (8%) cycles, and neuropathy in 5 (5%) of cycles. Twenty three patients had a platelet count < 100 x 10(9)/l prior to therapy: a greater than 50% decrease in platelet count over pre-therapy level occurred in 79% of first cycles, overt bleeding occurred in 4 (4%) of all cycles. Eleven of 29 (38%) patients achieved complete remission (CR), 4 of whom have relapsed after 5, 6, 9, and 12 months of remission. Two of 11 CR patients presented with RS without any prior CLL therapy. One patient had a partial remission. Thus the overall response rate was 12/29 (41%). Overall median survival was 10 months, 19 months in patients who achieved CR, 3 months in those who did not (p = 0.0008). A landmark analysis performed at 2 months from start of therapy comparing patients alive in CR versus patients alive but not in CR showed a median survival of 19 months versus 6 months, respectively (p 0.0017). In conclusion the hyper CVXD regimen has a relatively high response rate, significant toxicity and a moderate impact on survival in RS.
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PMID:Fractionated cyclophosphamide, vincristine, liposomal daunorubicin (daunoXome), and dexamethasone (hyperCVXD) regimen in Richter's syndrome. 1169 97

Two distinct morphological types of malignant lymphoma in the same patient occur mostly due to transformation of a low grade lymphoma (CLL) into a large--cell non-Hodgkin lymphoma (high-grade lymphoma). Later reports have brough evidence of a clonal relationship between CLL and supervening NHL. The Richter's syndrome was found to be more frequent in patients with CLL displaying either multiple chromosomal aberrations or monoclonal gammapaties. In the last two decades reports have evidenced the existence of two types of the Richter's syndrome: one, the "classical" as a terminal event in a long evolving CLL, the other "variant" as the first clinical manifestation of a previously unrecognized subclinical CLL. Aggressive chemotherapy of CLL play a role in transformation of CLL to Richter's syndrome.
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PMID:[Richter's syndrome (bimorphologic malignant lymphoma)]. 1196 89

Hodgkin's disease rarely develops in patients with B-chronic lymphocytic leukemia. Patients developing Hodgkin's disease after the diagnosis of chronic lymphocytic leukemia have been called the "Hodgkin's disease variant of Richter's transformation." We present a 62-yr-old man with a 17-mo history of chronic lymphocytic leukemia, who clinically and hematologically on remission was admitted to our clinic because of rapidly developing right cervical lymphadenopathy. He was diagnosed with lymph node biopsy as a mixed-cellularity Hodgkin's disease.
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PMID:Hodgkin's disease variant of Richter's transformation: a case report. 1218 Apr 78

Chronic lymphocytic leukemia (CLL) is an indolent B cell non-Hodgkin lymphoma (NHL) that may transform into diffuse large B cell lymphoma (DLBL). This transformation is referred to as Richter's syndrome or transformation. To analyze whether microsatellite instability (MSI) and DNA mismatch repair defects are associated with Richter's transformation, we have performed microsatellite analysis, mutational analysis of hMLH1 and hMSH2 genes and methylation status analysis of CpG island of the hMLH1 promoter on serial biopsy specimens from 19 patients with CLL. Ten cases of CLL showed no histologic alteration in the second biopsy, and nine cases of CLL underwent morphologic transformation to DLBL in the second biopsy. Using eight microsatellite loci, high level of MSI was associated with Richter's transformation in four cases of CLL, but none of the CLLs displayed this level of MSI without transformation. Mutations of the hMLH1 or hMSH2 genes were not detected in any of the lymphoma samples. In five cases of Richter's transformation the hMLH1 promoter was hypermethylated in both CLL and DLBL samples. Hypermethylation of the hMLH1 promoter associated with high-level of MSI in four cases, and low-level of MSI in one case. These results suggest that in certain cases of Richter's transformation the DNA mismatch-repair defect-initiated genetic instability may play a role in tumor progression.
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PMID:Microsatellite instability and hMLH1 promoter hypermethylation in Richter's transformation of chronic lymphocytic leukemia. 1259 41

Second malignancies are frequent complications in patients with chronic lymphocytic leukemia (CLL). Hodgkin's disease (HD) has been observed in approximately 0.5% of the patients with CLL and is known as Hodgkin's type Richter's syndrome (H-RS). We present a 64-year-old male patient with a familial history of CLL who developed H-RS in abdominal lymph nodes 6 years after CLL diagnosis and 18 months after treatment with cladribine (2-CdA) and cyclophosphamide. HD was diagnosed by fine needle aspiration. The disease was refractory to treatment with two courses of CHOP and three courses of ABVD chemotherapy. In the current literature we found case reports of only 6 patients with H-RS who were treated with fludarabine (FA) before transformation, and, to our knowledge the presented patient is the first to develop H-RS after treatment with 2-CdA combined with cyclophosphamide. He is also the first published patient with familial CLL in whom this complication developed.
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PMID:Hodgkin's type of Richter's syndrome in familial chronic lymphocytic leukemia treated with cladribine and cyclophosphamide. 1280 26

Richter's syndrome is defined by the occurrence of high-grade malignant lymphoma in the course of chronic lymphocytic leukemia (CLL). The prognosis is poor with a life expectancy of a few months. We report on the case of a 68-year old male who developed Hodgkin's disease 6 years after the diagnosis of stage A CLL was made. The patient received 3 cycles of combination chemotherapy according to the ABVD regimen, followed by radiotherapy. This treatment resulted in complete apparent remission of the two diseases, which was persistent after a follow-up of 14 years. The Hodgkin's disease variant of Richter's syndrome is exceptional with about 20 reported cases to date. Recent research indicates that lymphoma cells derive from leukemia. The prognosis appears far better than that for the classical non-Hodgkin Richter's syndrome.
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PMID:Hodgkin's disease variant of Richter's syndrome: complete remission of the both malignancies after 14 years. 1291 40


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