References

The Non-Hodgkin's Lymphoma Pathologic Classification Project National Cancer Institute sponsored study of classifications of non-Hodgkin's lymphomas. Cancer 449 2112-2135, 1982. 2. Harris NL, Jaffe ES, Stein H, et al. A revised European-American classification of lymphoid neoplasms a proposal from the International Lymphoma Study Group. Blood 84 1361-1392, 1994. 3. Harris NL, Jaffe ES, Diebold J, et al. World Health Organization classification of neoplastic diseases of the hematopoietic and...

Gmcsf

GM-CSF is a multilineage CSF because it stimulates the proliferation and differentiation of HPCs into neu- trophil, eosinophil, and monocyte colonies. GM-CSF functions in conjunction with other cytokines, ery-thropoietin and IL-3, to promote the proliferation and differentiation of erythroid and megakaryocytic progenitors, respectively. Administration of GM-CSF not only increases the number of monocytes but also increases the function of monocytes and macrophages including oxidative metabolism,...

Classification Of Aa Based On Etiology And Pathophysiologic Mechanisms

AA may have diverse causes that allow for clinically useful classification (Table 41.1). While iatrogenic AA is uncommon, it can be easily recognized. The most common is the idiopathic form of AA, and most parts of this chapter deal with this classical AA entity. Congenital bone marrow failure syndromes can evolve to AA they will not be discussed in detail here. Drugs and chemicals have been implicated as etiologic agents in AA for many decades. Benzene has served as a model chemical implicated...

Supportive Care

Infectious disease complications secondary to neu-tropenia are the primary cause of treatment-related morbidity and mortality in acute leukemia, and the risks seem to be especially high in older patients.44 Several studies have focused on preventing prolonged neutropenia in adults with ALL, with prophylactic use of granulocyte-stimulating growth factors.44 124 Two sequential studies of granulocyte colony-stimulating factor (G-CSF) use during induction therapy performed by the GMALL demonstrated...

Info

70-100 1 (oral morphine to parenteral fentanyl) PO, by mouth PR, by rectum SC, subcutaneous IV, intravenous ED, epidural IT - Intrathecal. PO, by mouth PR, by rectum SC, subcutaneous IV, intravenous ED, epidural IT - Intrathecal. with N-methyl-D-aspartate receptor antagonists) or by facilitating opioid analgesia, as occurs through tricyclic antidepressants (and enhanced monoamine release). Adjuvants are used for three reasons (1) a pain pathophysiology that is less responsive to opioids, such...

Conclusion

The reduction in toxicity due to ablative regimens is not likely to diminish by the development of new ablative programs. In fact, there are few examples of truly new ablative regimens. Rather, better understanding of the pathophysiology of RRTs will lead to new approaches for their prevention and treatment. Development of newer immunosuppressive regimens, such as the combination of tacrolimus or cyclosporine with mycophenolate mofetil, is likely to make a much greater impact on toxicity than...

Hhv6

Toxoplasma gondii CNS, pneumonia, disseminated disease reactivation of latent infection Strongyloides Enterocolitis, pneumonia, bowel stercoralis perforation, Gram-negative bacteremia Several pathogens require special comment. Legionellosis is uncommon but has been reported in the setting of HSCT.43 The disease may occur in the pretransplant or preengraftment periods, but is most common in the early engraftment period.43 A sentinel Intravascular devices Mouth and bowel with GVHD Mouth and bowel...

Chemosensitive Disease Is Required To Achieve Benefit From Hdtasct

Two randomized studies comparing full-course salvage chemotherapy (SC) administered alone with curative intent with short-course SC followed by HDT and ASCT are reported. The British National Lymphoma Investigation (BNLI) randomly assigned relapsed and primary refractory patients to either carmustine, etopo-side, cytarabine, and melphalan (BEAM) HDT followed by ASCT or up to three cycles of mini-BEAM with standard support.8 The German Hodgkin's Lymphoma Study Group (GHSG) randomly assigned...

Retrospective Studies Of Limited Treatment

In a recent review, Conners wrote, there is no definite evidence that LPHL is less curable than CHL . . .49 In another review, Diehl concluded, there is no rationale for a less intensive treatment of LPHL compared with CHL.39 Yet he suggested that these treatment strategies might be too intensive, particularly when late effects such as secondary malignancies and cardiac and pulmonary complications are taken into account. Several authors have suggested that the management of these patients...

Tubular Dysfunction

Free light chains (Bence Jones protein) play a crucial role in causing renal damage in myeloma patients. The nephrotoxicity of certain light chains has previously been demonstrated. Incubation of light chain with renal cortical tissue slides inhibited organic ion trans- Myeloma cast nephropathy Renal amyloidosis Hyperuricemia Immunoglobulin deposition Renal tubular acidosis diseases Hyperviscosity syndrome Myeloma cell infiltration Pyelonephritis port, gluconeogenesis, and ammonia formation,43...

Teratogenicity Of Chemotherapeutic Agents

All chemotherapeutic agents are theoretically teratogenic and mutagenic. Fetal malformation, intrauterine growth retardation (IUGR), spontaneous abortion, and fetal death may occur. The teratogenicity of any particular drug depends on the timing of exposure, the total dose, and the drug characteristics on placental transfer. Drugs with high lipid solubility, low molecular weight, and decreased plasma protein binding have greater tendency for placental transfer from mother to fetus....

Guidelines And Recommendations

The optimal management of patients with NLPHL is uncertain and controversial. The NCCN guidelines for patients with NLPHL are complex and distinctly different than for patients with CHL these recommendations are based on lower-level evidence including clinical experience, and are not always with uniform agreement among the panel members.62 For patients with CS IA NLPHL confined to the high neck (above the hyoid bone), the NCCN guidelines recommend IF or regional RT. For patients with CS I-IIA...

Effect Of Malignancy Treatment On Fertility

Post-treatment of Hodgkin's lymphoma in men with chemotherapy results in testicular germ cell aplasia and decreased libido.5 The seminiferous epithelium inside the testes is most sensitive to the detrimental effects of chemotherapy. Therefore, after treatment with gonadotoxic agents, patients may be rendered oligozoospermic or azoospermic. Because testosterone production by the Leydig cells remains unaffected, patients still develop normal secondary sexual charac-teristics.19 However, treatment...

Indwelling iv catheters

Tunneled catheters are subject to several types of infection exit site cellulitis, bacteremia with or without external signs, tunnel infection, and septic thrombophlebitis. The most common causative organisms are coagulase-negative staphylococci, but Staphylococcus aureus, Enterococcus, Gram-negative bacilli, other skin flora, yeast, and occasionally nontuberculous mycobac-teria also may be causative organisms. Decisions regarding catheter removal often must be made in the face of fever,...

Testicular Tissue Harvesting

Although spermatogenesis does not occur in prepuber-tal testes, and prepubertal testes do not produce mature spermatozoa, these do contain the diploid stem germ cells from which haploid spermatozoa can be derived. Therefore, testicular tissue can be harvested from a biopsy and stored either as a tissue section or as isolated germ cells, before cancer therapy. Following cure and on entering adulthood, this tissue can be thawed and used to produce offspring in either of the two ways the stored...

Maternal Physiologic Changes With Pregnancy

Several normal adaptive changes occur in the hemato-logic environment during pregnancy. Plasma volume increases by about 50 with only a 20-50 rise in red cell mass, resulting in a normocytic anemia.6 Inadequate hematopoiesis can result if iron and folate stores are not supplemented in advance. The normocytic anemia often becomes microcytic if iron metabolism cannot keep pace with fetal demand despite supplementation. Leukocytosis occurs during pregnancy, most likely due to increased levels of...

Chemotherapy In Combination With Wbxrt

Chemotherapy in combination with WBXRT, or combined modality therapy, has been used to treat PCNSL for over two decades. Initially, chemotherapy regimens used to treat systemic NHL were employed. Systemic administration of CHOP or CHOD (with dexametha-sone), either before or after WBXRT, has been studied in two phase II and one randomized phase III trial.26 38 39 In the phase II, trials the reported median survivals of 10 and 13 months were no better than historical controls using WBXRT alone,...

Established singleagent oral chemotherapy

In patients who have CMML with a significant myelo-proliferative component, high WBC count, or organomegaly, treatment with single-agent chemotherapy has been the standard of care. Oral agents such as busulfan, 6-mercaptopurine, hydroxyurea, and oral etoposide have been used empirically with some success, but a prospective randomized study conclusively determined the superiority of hydroxyurea over etopo-side in terms of overall survival in patients with mostly advanced proliferative disease...

Clinical Findings

Table 88.1 Clinical symptoms of hyperviscosity - Dizziness that can rarely lead to coma - Gingival and gastrointestinal hemorrhages or menorrhagia patients with HV due to WM have symptoms of visual disturbance, and up to 50 of these patients will demonstrate ocular changes.25-27 The percentage of retinal involvement in patients with HV due to other paraproteinemias is unknown, but such cases have been reported.28 29 In a recent study, Menke et al. examined 46 patients with WM by indirect...

Tumor Lysis

Tumor lysis syndrome is usually seen 1-5 days after the initiation of chemotherapy in patients with AML and high circulating blast counts.25 In response to chemotherapy, leukemic cells lyse, leading to hyper-phosphatemia, hyperkalemia (or hypokalemia), hypo-calcemia, an elevated lactate dehydrogenase (LDH), and hyperuricemia. Hyperuricemia results from breakdown of nucleotide precursors in leukemic cells to hypoxanthine and xanthine, and subsequent conversion to uric acid.25 Renal failure can...

Acknowledgment Stem Cell

Dr Friedberg is supported in part by a grant from the National Cancer Institute (CA102216). 14. Misgeld E, Germing U, Aul C, et al. Secondary myelodys-plastic syndrome after fludarabine therapy of a low-grade non-Hodgkin's lymphoma. Leuk Res 25 95-98, 2001. 15. Pedersen-Bjergaard J, Andersen MK, Christiansen DH Therapy-related acute myeloid leukemia and myelodysplasia after high-dose chemotherapy and autologous stem cell transplantation. Blood 95 3273-3279, 2000. 16. Friedberg JW, Fisher RI...

Acknowledgment Of Luekemia

This study was supported in part by the Lymphoma Foundation and Victoria's Smile Foundation. 14. Ishii E, Mara T, Ohkubo K, et al. Treatment of childhood acute lymphoblastic leukemia with intermediate-dose cytosine arabinoside and adriamycin. Med Pediatr Oncol 14 73, 1986. 15. Kantarjian HM, Estey EH, Plunkett W, et al. Phase I-II clinical and pharmacologic studies of high-dose cyto-sine arabinoside in refractory leukemia. Am J Med 81 387, 1986. 16. Capizzi RI, Poole M, Cooper MR, et al....

Presentation

Most patients with AA present with symptoms of anemia or bleeding due to deficient red blood cell and platelet production. Infection associated with low neutrophil numbers is not a common initial presentation. Often, AA is uncovered incidentally patients may remain asymptomatic for a long time due to the latent onset of anemia. Consequently, the latency period and the onset of the disease are difficult to determine. In some cases, when AA is discovered early, a follow-up blood examination will...

Informed Consent

Informed consent is routinely a prerequisite for the procedures of UCB banking. Prenatal efforts are often directed at recruiting potential donors. Information regarding the risks and benefits of the collection, storage, and potential uses of UCB are explained to parents of prospective donors. By convention, the mother must consent by proxy as UCB is technically of fetal Juvenile chronic myelogenous leukemia Hurler's syndrome Myelodysplastic syndrome Adrenoleukody-strophy Blackfan-Diamond...

Cytoreductive agents

Hydroxyurea and busulfan are the agents most frequently reported as treatment choices in patients with diseases resembling aCML.62 Both can be used either on a daily schedule with the dose adjusted to the desired leukocyte blood count, or alternatively, on a cyclical schedule aiming initially for resolution of systemic symptoms and reduction of the leukocyte count. Due to its more rapid onset of action and lack of delayed effect on the bone marrow, hydroxyurea is more suitable for continuous,...

Platelet Transfusion

As early as 1910, platelet transfusions were shown to have a beneficial effect in bleeding patients.18 In the 1960s, the development of plastic blood collection and storage bags allowed the ready concentration and storage of platelets. In 1964, investigators at the National Cancer Institute reported the efficacy of platelet transfusions in patients undergoing therapy for leukemia.18 Thrombocytopenia or decreased platelet function are common complications in patients with hemato-logic...

Acknowledgments

The authors are supported by grants from the Leukemia Lymphoma Society (VK, SN), the NIH P01 CA05826 (CCPP) (SN), and the Rosemary Breslin Research fund. The authors thank Heather Hewitt and Ellie Park, for assisting in the preparation of this work, our colleagues at Memorial Sloan-Kettering Cancer Center, and the Myelodysplastic Syndrome Foundation. 7. Telfer PT, et al. Hepatic iron concentration combined with long-term monitoring of serum ferritin to predict complications of iron overload in...

Thrombocytopenia

A low platelet count often is present in a person with acute leukemia, either from bone marrow replacement or from the bone marrow suppressive effects of the leukemia itself from platelet consumption as a sequela of the disease or infection or from the myelosuppres-sive effects of antileukemic chemotherapy. The incidence of serious spontaneous hemorrhage increases when the platelet count falls below 10,000 mm3, and this should be the threshold for platelet transfusions in the absence of...

Agnogenic Myeloid Metaplasia Ammmyelofibrosis With Myeloid Metaplasia

AMM (also known as idiopathic MMM), is characterized initially by a hypercellular bone marrow, extramedullary hematopoiesis, splenomegaly, and a leukoerythroblastic peripheral blood picture. The disease can be defined as the causally unknown (agno-genic) proliferation of hematopoietic cells (myeloid cells) in organs or tissues that are not usually involved in blood cell formation (metaplasia). Extramedullary hematopoiesis is a term meaning that this blood cell formation occurs outside the...

Demethylating Agents

DNA methylation is a normal mechanism of gene expression regulation, and abnormal DNA methyla-tion patterns that can interfere with the expression of genes used for growth, differentiation, and survival are seen in MDS. It has been known for some time that hypomethylating drugs can promote in vitro cellular differentiation,50 and that this occurs at doses much lower than those needed for maximal cytotoxic effect. The clinical trials using the DNA demethylating agents 5-azacytidine (azacitidine,...

Incidence And Clinical Presentation

The incidence of polycythemia vera (PV) is slightly higher in men than in women (2.8 vs 1.3 cases per 100,000 per year).1 The mean age at diagnosis is 62 years (range, 20- 85).2 The clinical presentation of patients with PV can be nonspecific, and may include headaches (48 ), weakness (47 ), dizziness (43 ,) pruritus (43 ), and excessive sweating (33 ). Most patients are asymptomatic and come to a hematologist's attention based on a routine complete blood count (CBC). Erythromelalgia (burning...

Vinca Alkaloids

Vincristine, vinblastine, vindesine, vinorelbine The vincas are natural compounds originally derived from the periwinkle plant. Two of these compounds, vincristine and vinblastine, are usedin the treatment of a variety of hematological malignancies, including lymphocytic leukemia, lymphoma, Hodgkin's disease, and myeloma. Vinca alkaloids are known as spindle poisons, because of their ability to inhibit the assembly of microtubules. This targeting of microtubules is thought to be one of the most...

Summary

Hematologic malignancies have a cluster of symptoms that differ in degree from solid tumors. Fatigue, depression, delirium, treatment-related mucositis, and treatment-related pain are common symptoms. These symptoms can occur at early stages of disease, can persist through remission, and will require both nonpharmaco-logic and pharmacologic management. Involvement of palliative therapy early in the course of disease can minimize suffering from multiple symptoms while the patient undergoes...

Interferon a

There are no prospective data on the efficacy of interferon a IFN-a in treating aCML. IFN-a has been used, however, in Ph-negative CML, and a retrospective analysis of some of these patients revealed the absence of the BCR-ABL abnormality, rendering the diagnosis similar to aCML. In one report, two of six patients with BCR-ABL-negative CML achieved a CR, while four patients failed to respond.63 In another study involving Ph-negative CML, 21 patients were treated with a daily subcutaneous dose...

Methods Of Sct Myeloablative regimens

A number of myeloablative regimens have been used for SCT in AML. The two most common were derived Table 35.2 Risk stratification of acute myeloid leukemia Favorable Age lt 60 WBC lt 100,000 De novo leukemia t 15 17 t 8 21 inv16 or t 16 16 absence of high risk cytogenetic abnormalities or FLT3-ITD Antecedent hematologic condition e.g., myelodysplasia or prior chemotherapy radiation Deletions monosomies of chromosome 3, 5, 7, or complex karyotype gt 3 per SWOG or gt 5 per MRC , t6 9, 11q23...

Idarubicin For Myeloma

Monconduit M, Menard JF, Michaux JL, et al. VAD or VMBCP in severe multiple myeloma. The Groupe d'Etudes et de Recherche sur le Myelome GERM . Br J Haematol 80 2 199-204, 1992. 33. Barlogie B, Kyle RA, Anderson KC, et al. Comparable survival in multiple myeloma MM with high dose therapy HDT employing MEL 140 mg m2 TBI 12 Gy autotransplants versus standard dose therapy with VBMCP and no benefit from interferon IFN maintenance results of intergroup trial S9321. Session type oral session...

Do Patients With Primary Refractory Disease Have A Suboptimal Outcome With Asct

While response to SC is the major selection criteria to proceed to ASCT, other prognostic factors also predict for long-term FFTF in patients with relapsed and refractory HL. Some groups have suggested that patients with primary refractory disease do less well than those patients who achieve an initial remission to front-line therapy. There are conflicting registry data in this regard. The North American Autologous Blood and Bone Marrow Transplant Registry ABMTR reported on a series of 122...

Blood And Bone Marrow Changes

Anemia is present in 60-80 of patients at presentation.1-5 It is usually mild to moderate in severity and is normocytic, normochromic in morphology. The anemia has the characteristics of anemia of chronic inflammation formerly anemia of chronic disease with low serum iron, decreased transferrin saturation, and elevated ferritin. The reticulocyte count is low, reflecting a hypoproliferative state. A bone marrow examination commonly reveals erythroid hypoplasia and a varying degree of myeloma...

Therapy of relapse DLI and imatinib

ALL in relapse after transplantation is rarely salvaged by donor lymphocyte infusion. This observation is consistent but confusing, since there does appear to be a measurable GVL response in primary transplantation. Less than 20 of patients will achieve a remission to DLI alone52,53 however, this number may be increased with concomitant chemotherapy. Despite this, remissions are rarely durable. Imatinib has been used as therapy for relapse after transplantation of ALL54 however, the majority of...

Introduction

Most of the 15,000 people per year in the United States who develop multiple myeloma MM 1 require some form of treatment at the time of diagnosis. Combination regimens incorporating alkylating agents, corticosteroids, and or anthracyclines have been used for decades, with little overall progress during that time in terms of survival or cure rate.2' 3 High-dose chemotherapy with autologous stem cell support HDC ASCS , shown in some studies to offer a modest survival benefit, is nonetheless...

Aggressive Transformation

There is much confusion about aggressive transformation in chronic lymphocytic leukemia CLL . Unlike chronic myeloid leukemia where such a transformation is the rule, in CLL it is rare. Moreover, transformation to acute lymphoblastic leukemia ALL , although reported in the literature,1 is a myth based on a misunderstanding of the nature of CLL. We can, however, recognize different ways in which CLL can transform Table 27.1 . In 1928, Maurice Richter described an aggressive lymphoma occurring in...

Cytologyhistopathology

Pseudofollicular Centers

The key diagnostic feature of CLL relies on a careful examination of the peripheral blood smear. In a typical case, there is a lymphocytosis of small, round, mature-appearing lymphocytes with scant amounts of cytoplasm and mature chromatin. Typically, the chromatin has a characteristic clumped appearance with absent nucleoli. Disrupted lymphocytes, known as smudge cells, are a common finding in CLL Figure 22.1 . A lym-phocytosis of 5 X 109 lymphocytes L has been a mandatory part of the...

Deletion Of Chromosome 13q

Deletion Chromosome Myeloma

Deletion of chromosome 13q is the most common recurrent chromosomal abnormality in myeloma. It is detected in 15-20 of myeloma by CC,21 50 of cases by interphase FISH in newly diagnosed myeloma patients,22 and 30-45 of cases of MGUS.23'24 The prognostic significance of loss of 13q identified by FISH alone has been the subject of considerable debate. Several studies based on molecular cytogenetics have shown that this abnormality is strongly associated with an unfavorable prognosis.2325-32 It...

Monoclonal Antibodies For Treatment Of Classical Hodgkins Lymphoma

Among the different target antigens on Reed-Sternberg cells, CD30 seems to be the most promising, since it is expressed at very high levels.75 So far, two anti-CD30 MoAbs have been developed, the humanized SGN-3076 and the fully human MDX-60.77The SGN-30 chimeric anti-CD30 antibody has demonstrated antitumor activity in preclinical models of Hodgkin's lymphoma and anaplastic large cell lymphoma. In a phase I single-dose trial, SGN-30 MoAb showed minimal toxicity with doses from 1 to 15 mg kg,...

The Morphologic Diagnosis Of Multiple Myeloma

The morphologic diagnosis of multiple myeloma and its distinction from reactive plasmacytosis relies on both the quantity of plasma cells seen and the qualitative plasma cell abnormalities, as previously mentioned. In general terms, more than 30 of plasma cells in a marrow aspirate smears constitute a major diagnostic criterion for multiple myeloma, although such a percentage may occur in other conditions, such as rheumatic disorders, inflammatory reactions of the bone marrow, and in...

Aml Transplant Prognosis

Although many clinical and pathologic features of AML have prognostic relevance, only a few prognostic factors are universally agreed upon, validated, and impact clinical practice. Nonetheless, newer biologic markers of prognosis are likely to supplant older clinical markers in the near future. As discussed further, and in greater detail in Chapter 5, age greater than 60 years invokes many adverse features that make separation of age from other poor prognostic markers difficult. Nonetheless,...

Future Directions In Therapy For Advancedphase

The success of imatinib will clearly result in fewer patients with CML progressing to the advanced phases. Is blastic phase CML a disease of the past Further observational time will let us know what percent of patients with CML will be cured with imatinib. Although leukemia cells resistant to imatinib have been detected in patients with CML, this has mostly occurred in advanced-phase patients who received imatinib for the first time, having received prior treatment with other agents.47 The...

Myelodysplastic Syndrome Mds And Acute Myeloid Leukemia Aml Incidence And Risk Factors

Historically, myelodysplastic syndrome MDS and secondary acute myelogenous leukemia have been recognized as significant complications of alkylating-agent-based chemotherapy for both indolent NHL and Hodgkin's lymphoma.1112 Topoisomerase inhibitors13 and purine analogs14 also clearly contribute to the risk of AML and MDS. With the recent introduction of alternative chemotherapy and immunotherapeutic modalities, autologous stem cell transplantation ASCT and radioimmunotherapy represent the...

Laboratory Abnormalities In Hyperleukocytosis

Pseudohyperkalemia can be present in patients with a high white blood count secondary to breakdown of white cells in vitro with subsequent release of potas-sium.2 Other spurious laboratory data that can be seen in association with hyperleukocytosis include a falsely elevated platelet count secondary to white cell fragments , pseudohypoxemia secondary to oxygen consumption by leukocyte cells , falsely prolonged coagulation tests, and pseudohypoglycemia.421-24 Pseudohypoxemia and...

Immune Stimulation And Myeloma Chronic infections and eczema

Many medical conditions associated with chronic stimulation of the immune system, such as repeated infections, allergic conditions, or autoimmune disease, have been reported to increase the risk of MM. In a case-control study, past history was abstracted from medical records for leukemia, n 299 non-Hodgkin's lymphoma NHL , n 100 and MM, n 175 patients, and matched with 787 controls. Prior histories of eczema and musculoskeletal conditions were associated with higher risk for MM with no role...

Targeting Cd52 On Hcl Cells With Alemtuzumab

CD52 is a 12-amino-acid glycoprotein that is present on lymphocytes at up to 450,000 sites cell.48'49 It is also present on monocytes, macrophages, eosinophils, and the male reproductive tract.5051 Quigley et al. at the Scripps Clinic recently reported that in nine cases of classic HCL and one of HCLv, all patients expressed CD52 on 92-100 of the HCL cells.52 Fietz et al. reported recently that a patient with HCL and shortlived or poor responses to cladribine, interferon, splenectomy, and...

Differential Diagnosis

Lymphocytosis Differential Diagnosis

A malignant lymphoproliferative disorder should be suspected when the absolute lymphocyte count is greater than 5000 lymphocytes L. The differential diagnosis for patients with lymphocytosis, lym-phadenopathy, and or organomegaly includes numerous malignant lymphoproliferative disorders Table 23.1 . These include CLL, PLL, adult peripheral T-cell lymphoma, natural killer cell leukemia, mantle cell lymphoma, marginal zone lymphoma nodal, extran-odal, or splenic , hairy cell leukemia, SLL,...

With Bcrablderived Peptide Vaccines

As discussed above, many clinicians still consider allo-geneic stem cell transplantation to be the gold standard for curative therapy in CML by virtue of the long-term survival achieved and the ability of this modality to render the patient BCR ABL negative using PCR-based assays. The importance of the immunologic properties of the graft has received a great amount of attention, with attempts to recapitulate a GvL effect while separating it from the side effects of conventional transplantation...

Treatment Of Small And Mediumsized Pleomorphic Tcell Lymphoma And Cd30 Large Tcell Lymphoma

Patients with CD30 LTCL or small medium-sized PTCL usually present with solitary, localized, or generalized plaques, nodules, or tumors without spontaneous regression.96 Both entities do not express CD30 with absence of a Th2 cytokine profile in CD30 LTCL.97 In CD30- LTCL, large cells comprise over 30 of the dermal infiltrate and might resemble classical MF undergoing large cell transformation. Multiagent systemic chemotherapy is recommended in most cases, with radiotherapy limited to localized...

Cd5 B Cells And The Possible Role Of Clonal Lymphocytes Of Unknown Significance In

CD5 is a highly conserved single-chain 67-kDa transmembrane glycoprotein containing three scavenger receptor cysteine-rich SRCR domains. CD5 expression is found on all human T cells, but only on a subset of B cells. Despite its sequence conservation, CD5 expression in T cells and B cells varies widely from species to species in some, all B cells are CD5 positive. Studies on CD5-deficient mice have shown that CD5 functions as a negative regulator of B-cell...

Agriculture

Agricultural work predominantly farming is an occupation that has been most frequently associated with MM.81 Early reports from Iowa farmers in the 1970s showed a significantly higher mortality rate for MM and leukemia.82 A recent update of these data suggest that association between farmers and MM is weaker than initially reported.983 84 Similar results were reported from Sweden. There were 568 MM cases among 254,417 men working in agriculture, with an estimated RR of 1.20 95 CI 1.09-1.33 .85...

Histologic Transformation Of Follicular Lymphoma

Patients with a follicular lymphoma have a relatively constant risk over time of transforming into a more aggressive histology, most often a diffuse large B-cell lymphoma.16119120 The frequency of this occurrence varies among series from fewer than 20 16 to at least 30 .119 The difference among studies reflects a number of factors, including the duration of follow-up, the definition of what is called transformed lymphoma, and the method of surveillance. In studies in which patients had lymph...

Making The Diagnosis

On a routine complete blood count CBC , most patients with AML are anemic and thrombocytopenic.78 The white blood count is variable, with 20 of patients having white blood counts less than 5000 mm3 and 20 of patients with white blood counts greater than 100,000 mm3.78 High white blood counts and hyperleukocytosis are more common in the monocytic leukemias.36 79 Although blasts are usually present in the peripheral blood, a subset of patients present with aleukemic leukemia. A review of the...

Cytogenetic Remission

Cytogenetic remission is defined as a reduction in the number of identifiable Philadelphia Ph chromosomes by standard metaphase karyotypic analysis. The degree of reduction determines the completeness of remission, and this degree of reduction has prognostic significance with regard to survival. The importance of achieving a major or complete cytogenetic remission CCR is highlighted by the results of several randomized clinical trials comparing interferon-a to chemotherapy such as hydroxyurea....

Aml With Rearrangements Of Band 11q23 And The Mll Gene

This category represents approximately 4 of cases of adult AML, but rearrangements involving band 11q23 and the MLL gene also known as ALL1, HRX, and HTRX are three to four times more common in children with AML, being especially frequent among infants aged 12 months or less, 43-58 of whom carry an 11q23 MLL abnormality.13 At both the cytogenetic and molecular genetic level, AML with the 11q23 MLL rearrangements is extremely heterogeneous. Well over 30 different balanced chromosome...

Definition Of Remission

In broad terms, a complete remission CR in AML is defined as the inability to detect leukemia, using standard tests i.e., peripheral blood smears, bone marrow biopsy and aspirate, and flow cytometry to the greatest extent possible. It does not indicate or imply cure, though patients may mistake the two and assume that CR and cure are equivalent.1 Another way of approaching this concept is to consider the following thought experiment. When a patient presents with AML, he or she presumably has...

Posttransplant Consolidation Maintenance Therapy

HDC with AHCT can cure nearly half of NHL patients with chemosensitive disease and about 30 of patients with primary refractory disease. However, relapse still accounts for the majority of deaths in this patient population with most relapses occurring in sites of previous bulk disease. IFRT as adjuvant therapy posttransplantation is a strategy that can reduce this risk. Advantages for administering IFRT posttransplantation include the ability to tailor radiation dose based on residual disease...

Treatment Of Malt Lymphoma

H. pylori eradication in gastric MALT lymphoma The regression of gastric MALT lymphoma after antibiotic eradication of H. pylori was first reported in 1993 by Wotherspoon and colleagues, who described the efficacy of antibiotic therapy in six patients with superficially invasive gastric MALT lymphoma.55 Several groups thereafter confirmed the efficacy of antibiotics in inducing apparently durable lymphoma remissions in 60-100 of patients with localized H. Pylori-positive gastric MALT...

Cytogenetics And Molecular Cytogenetics

Regular cytogenetic analysis of CLL is confounded by the low proliferative rate of the bulk of the cells and the presence of residual normal T cells. Comprehensive analysis has been performed by a number of dedicated centers worldwide, where conditions for CLL cytogenetics have been optimized. From these studies it is clear that, unlike the related diseases of mature B cells, such as follicular lymphoma or MCL, there is no obvious consistent cytogenetic lesion in CLL. A number of recurrent...

Primary Mediastinal Bcell Lymphoma

Primary mediastinal B-cell lymphoma PMBL is a distinct subtype of diffuse large cell lymphoma originating from thymic B cells. It was first described nearly 25 years ago5960 and is now recognized as a unique entity based on clinical, immunophenotypic, and genotypic features. PMBL accounts for approximately 5 of all aggressive lymphomas and affects mainly young adults. The median age at presentation is in the third decade, with a slight female predilection. Most patients present with significant...

Molecular Anatomy Of The Bcrabl Fusion

Bcr Abl Breakpoints

The breakpoints in ABL are spread over a large genomic region and may occur 5' of ABL exon Ib, between ABL exons Ia and II or, most frequently, between the two alternative first ABL exons Figure 17.2 reviewed in Ref. 36 . Despite this variation, the ABL portion in the fusion mRNA and protein is usually constant, encompassing ABL exons 2-11. This is thought to be the result of posttranscriptional processing of the primary transcript. In contrast, the breakpoints in BCR localize to three distinct...