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Hemato, Volume 3, Issue 4 (December 2022) – 13 articles

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8 pages, 4516 KiB  
Case Report
Abdominal Lymphadenopathy: Hypothesize Cat-Scratch Disease and Avoid Abdominal Excisional Biopsy
by Valeria Filipponi, Stefania Trasarti, Francesca Maccioni, Maddalena Zippi, Ludovica Busato, Francesca Arienzo, Mario Biglietto, Paolina Saullo, Carla Giordano and Roberto Caronna
Hemato 2022, 3(4), 771-778; https://doi.org/10.3390/hemato3040052 - 7 Dec 2022
Cited by 1 | Viewed by 1650
Abstract
The finding of lymphadenopathy is usually the consequence of a benign infection, although a neoplastic origin must always be excluded. Through a careful anamnesis, physical examination, and serological tests several differential diagnoses are frequently possible. Nevertheless, sometimes an excisional biopsy of superficial lymph [...] Read more.
The finding of lymphadenopathy is usually the consequence of a benign infection, although a neoplastic origin must always be excluded. Through a careful anamnesis, physical examination, and serological tests several differential diagnoses are frequently possible. Nevertheless, sometimes an excisional biopsy of superficial lymph nodes is required, which is the best means to reach a definitive diagnosis. More concerns arise when lymphadenopathy is only abdominal/retroperitoneal: percutaneous biopsy is often inconclusive and the excisional node biopsy becomes a surgical procedure, certainly indicated in case of malignancy but avoidable in case of inflammatory diseases. We present the case of a 30-year-old man with a deep iliac lymphadenopathy who was evaluated at the Hematological Unit of Sapienza University of Rome. The enlargement of an iliac lymph node is quite unusual for an infectious disease. Although symptoms such as pain, fever, and chills suggested it was the case, cat-scratch disease was not hypothesized. Radiological investigations did not exclude a malignant disease and a laparoscopic excisional biopsy was scheduled, but the slight improvement of his spontaneous symptoms suggested a careful follow-up. Given the lack of disappearance of lymphadenopathy, the lack of diagnosis, and an ipsilateral superficial (inguinal) lymph node with similar ultrasonographic and radiological features, the patient underwent biopsy, which disclosed a diagnosis of cat-scratch disease, avoiding more invasive surgical procedures. Full article
(This article belongs to the Section Non Neoplastic Blood Disorders)
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13 pages, 1061 KiB  
Review
Bing–Neel Syndrome: Update on Diagnosis and Treatment
by Evangeline Y. Wong, Shirley D’Sa, Monique C. Minnema, Jorge J. Castillo and Dipti Talaulikar
Hemato 2022, 3(4), 758-770; https://doi.org/10.3390/hemato3040051 - 29 Nov 2022
Cited by 1 | Viewed by 7325
Abstract
Bing–Neel syndrome (BNS) is a rare neurological complication of Waldenström macroglobulinaemia. We highlight key issues in clinical presentation, diagnosis, and treatment while focusing on new and emerging therapies available for patients diagnosed with BNS. It is anticipated that further development of Bruton Tyrosine [...] Read more.
Bing–Neel syndrome (BNS) is a rare neurological complication of Waldenström macroglobulinaemia. We highlight key issues in clinical presentation, diagnosis, and treatment while focusing on new and emerging therapies available for patients diagnosed with BNS. It is anticipated that further development of Bruton Tyrosine Kinase (BTK) inhibitors and less toxic chemoimmunotherapies will improve treatment delivery and response. Full article
(This article belongs to the Special Issue Waldenström Macroglobulinaemia and Related Conditions)
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16 pages, 648 KiB  
Review
TP53 Mutant Acute Myeloid Leukemia: The Immune and Metabolic Perspective
by Federico Zingarelli, Letizia Zannoni and Antonio Curti
Hemato 2022, 3(4), 742-757; https://doi.org/10.3390/hemato3040050 - 15 Nov 2022
Viewed by 5349
Abstract
TP53 mutated/deleted acute myeloid leukemia (AML) stands out as one of the poorest prognosis forms of acute leukemia with a median overall survival not reaching one year in most cases, even in selected cases when allogenic stem-cell transplantation is performed. This aggressive behavior [...] Read more.
TP53 mutated/deleted acute myeloid leukemia (AML) stands out as one of the poorest prognosis forms of acute leukemia with a median overall survival not reaching one year in most cases, even in selected cases when allogenic stem-cell transplantation is performed. This aggressive behavior relies on intrinsic chemoresistance of blast cells and on high rates of relapse. New insights into the biology of the disease have shown strong linkage between TP53 mutant AML, altered metabolic features and immunoregulation uncovering new scenarios and leading to possibilities beyond current treatment approaches. Furthermore, new targeted therapies acting on misfolded/dysfunctional p53 protein are under current investigation with the aim to improve outcomes. In this review, we sought to offer an insight into TP53 mutant AML current biology and treatment approaches, with a special focus on leukemia-associated immune and metabolic changes. Full article
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11 pages, 264 KiB  
Article
IgM-Related Immunoglobulin Light Chain (AL) Amyloidosis
by Shayna Sarosiek, Andrew R. Branagan, Steven P. Treon and Jorge J. Castillo
Hemato 2022, 3(4), 731-741; https://doi.org/10.3390/hemato3040049 - 15 Nov 2022
Viewed by 3122
Abstract
Waldenström macroglobulinemia (WM) is a rare lymphoplasmacytic disorder characterized by an IgM paraprotein. The clinical presentation of WM varies and can include common manifestations such as anemia and hyperviscosity, in addition to less common features such as cryoglobulinemia, IgM-related neuropathy, and immunoglobulin light [...] Read more.
Waldenström macroglobulinemia (WM) is a rare lymphoplasmacytic disorder characterized by an IgM paraprotein. The clinical presentation of WM varies and can include common manifestations such as anemia and hyperviscosity, in addition to less common features such as cryoglobulinemia, IgM-related neuropathy, and immunoglobulin light chain (AL) amyloidosis. Amyloidosis is a protein-folding disorder in which vital organ damage occurs due to the accumulation of misfolded protein aggregates. The most common type of amyloidosis in patients with an IgM paraprotein is AL amyloidosis, although other types of amyloidosis may occur. IgM-related amyloidosis has distinct clinical features when compared with other subtypes of AL amyloidosis. This review highlights the diagnostic criteria of IgM-related AL amyloidosis, as well as the clinical characteristics and treatment options for this disorder. Full article
(This article belongs to the Special Issue Waldenström Macroglobulinaemia and Related Conditions)
13 pages, 298 KiB  
Review
Update in Childhood Chronic Myeloid Leukemia
by Fiorina Giona and Simona Bianchi
Hemato 2022, 3(4), 718-730; https://doi.org/10.3390/hemato3040048 - 5 Nov 2022
Cited by 2 | Viewed by 2519
Abstract
Chronic myeloid leukemia (CML) in childhood represents only 3% of newly diagnosed pediatric leukemia. The diagnostic hallmark of CML is the Philadelphia (Ph) chromosome, which derives from the fusion of the ABL1-oncogene located on chromosome 9 to the breakpoint cluster region ( [...] Read more.
Chronic myeloid leukemia (CML) in childhood represents only 3% of newly diagnosed pediatric leukemia. The diagnostic hallmark of CML is the Philadelphia (Ph) chromosome, which derives from the fusion of the ABL1-oncogene located on chromosome 9 to the breakpoint cluster region (BCR) gene on chromosome 22, resulting in a constitutively dysregulated ABL1 tyrosine kinase, either as 210 kDa or 190 kDa. Depending on the localization of the breakpoint site within the major BCR region, the majority of CML patients exhibit transcripts with either the b3a2 or b2a2 junction, or both. Several questions are still open with regard to childhood CML, especially concerning the biologic and clinical features of the disease, and the treatment of choice for pediatric patients with CML. Moreover, over the last few years, several tyrosine kinase inhibitors (TKIs) have been available for children and adolescents with CML, and current clinical practice investigates what the effective and optimal doses of TKIs are in these two categories of patients. The use of TKIs in pediatric patients with CML has also opened up questions on the following items: (1) the long-term effects of these drugs on children; (2) the management of pediatric CML forms resistant or intolerant to TKIs; (3) the monitoring of disease outcomes during treatment; (4) and the right timing to discontinue therapy. Despite the efficacy of TKIs also in the pediatric population, the potential late adverse effects, and the drug resistance, leave open the possibility of allogeneic hematopoietic stem cell transplantation as a treatment option in pediatric CML. Published data and personal experiences regarding these issues will be analyzed and discussed. Full article
14 pages, 305 KiB  
Review
First-Line Treatment of Waldenström’s Macroglobulinaemia: Considerations Based on the Dutch National Guideline
by Karima Amaador, Marie José Kersten, Hein P. J. Visser, Laurens Nieuwenhuizen, Roelandt F. J. Schop, Martine E. D. Chamuleau, Gerjo A. Velders, Monique C. Minnema and Josephine Mathilde Iris Vos
Hemato 2022, 3(4), 704-717; https://doi.org/10.3390/hemato3040047 - 26 Oct 2022
Viewed by 2720
Abstract
Waldenström macroglobulinemia (WM) is a rare B-cell Non-Hodgkin Lymphoma. There are only few prospective randomized clinical trials to guide treatment recommendations and there is no international consensus on a preferred first line treatment approach. In the recently revised Dutch guideline for WM, we [...] Read more.
Waldenström macroglobulinemia (WM) is a rare B-cell Non-Hodgkin Lymphoma. There are only few prospective randomized clinical trials to guide treatment recommendations and there is no international consensus on a preferred first line treatment approach. In the recently revised Dutch guideline for WM, we describe recommendations for practice based as much as possible on the known data. Here, we summarize the considerations for first-line treatment based on these Dutch guidelines. Available evidence is summarized, including efficacy and toxicity data. Combinations of Rituximab with chemotherapy, proteasome inhibition or BTK-inhibition are all valid first line treatment options. The Dutch WM working group considers Dexamethasone/Rituximab/Cylofosfamide (DRC) a suitable first-line treatment for many WM patients, given the efficacy, the relatively mild toxicity profile and the extensive experience with this regimen. However, the long-term toxicities of DRC are unclear and need further clarification. Other regimens such as R-bendamustine, R-Bortezomib-dexamethason are also effective options, however with specific toxicities. BTK-inhibitors are not a preferred option in first line for most patients in the Dutch WM guidelines because of the need for longterm treatment and toxicities. Based on patient preferences research, future clinical trials should focus on effective fixed-duration regimens with non-cytotoxic therapies that have a favorable toxicity profile. Further development of (combinations with) BCL-2 inhibititors, novel proteasome inhibitors and BTK-inhibition could be interesting. In addition T-cell-directed treatments including bispecific antibodies as a monotherapy or combined with other novel agents deserve further study in WM. Full article
(This article belongs to the Special Issue Waldenström Macroglobulinaemia and Related Conditions)
15 pages, 576 KiB  
Review
Should Patients with Waldenström Macroglobulinemia Receive a BTK Inhibitor as Frontline Therapy?
by Marina Deodato, Anna Maria Frustaci, Giulia Zamprogna, Giulia Cotilli, Roberto Cairoli and Alessandra Tedeschi
Hemato 2022, 3(4), 689-703; https://doi.org/10.3390/hemato3040046 - 23 Oct 2022
Viewed by 2098
Abstract
Waldenström Macroglobulinemia (WM) is a rare indolent lymphoma with heterogeneous clinical presentation. As there are no randomised trials suggesting the best treatment option in treatment-naive patients, guidelines suggest either rituximab-combining regimens or BTK-inhibitors (BTKi) as feasible alternatives. Several factors play in the decision-making [...] Read more.
Waldenström Macroglobulinemia (WM) is a rare indolent lymphoma with heterogeneous clinical presentation. As there are no randomised trials suggesting the best treatment option in treatment-naive patients, guidelines suggest either rituximab-combining regimens or BTK-inhibitors (BTKi) as feasible alternatives. Several factors play in the decision-making process: patients’ age and fitness, disease characteristics and genotype. Chemoimmunotherapy (CIT) represents a fixed-duration, less expensive and effective option, able to achieve prolonged time-to-next treatment even in patients with unfavourable genotypes. Immunosuppression and treatment-related second cancers may represent serious concerns. Proteasome-inhibitor-based regimens are effective with rapid disease control, although bortezomib-related neuropathy discourages the choice of these agents and treatment schedules may not be easily manageable in the elderly. BTKi have demonstrated high rates of response and prolonged survival together with the convenience of an oral administration and limited cytopenias. However, outcomes are impacted by genotype and some concerns remain, in particular the continuous drug exposure that may result in extra-haematological complications and drug resistance. Although next-generation BTKi have improved treatment tolerance, the question whether BTKi should be offered as frontline therapy to every patient is still debated. Giving fixed-duration schedule, prolonged time-to-next treatment and outcomes independent of genotype, CIT is still our preferred choice in WM. However, BTKi remain a valuable option in frail patients unsuitable for CIT. Full article
(This article belongs to the Special Issue Waldenström Macroglobulinaemia and Related Conditions)
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26 pages, 768 KiB  
Review
Polyneuropathy Associated with IgM Monoclonal Gammopathy; Advances in Genetics and Treatment, Focusing on Anti-MAG Antibodies
by Johannes P. M. van de Mortel, Shirley D’Sa, Alexander F. J. E. Vrancken, Nicolette C. Notermans, Josephine M. I. Vos and Monique C. Minnema
Hemato 2022, 3(4), 663-688; https://doi.org/10.3390/hemato3040045 - 17 Oct 2022
Cited by 1 | Viewed by 5254
Abstract
With increasing age, the chances of developing either MGUS or polyneuropathy increase as well. In some cases, there is a causative relationship between the IgM M-protein and polyneuropathy. In approximately half of these cases, IgM targets the myelin-associated glycoprotein (MAG). This results in [...] Read more.
With increasing age, the chances of developing either MGUS or polyneuropathy increase as well. In some cases, there is a causative relationship between the IgM M-protein and polyneuropathy. In approximately half of these cases, IgM targets the myelin-associated glycoprotein (MAG). This results in chronic polyneuropathy with slowly progressive, predominantly sensory neurological deficits and distally demyelinating features in nerve conduction studies. Despite the disease being chronic and developing slowly, it can cause considerable impairment. We reviewed English medical publications between 1980 and May 2022 on IgM gammopathy-associated polyneuropathy, with special attention to studies addressing the pathophysiology or treatment of anti-MAG polyneuropathy. Treatment options have been limited to a temporizing effect of intravenous immunoglobulins in some patients and a more sustained effect of rituximab but in only 30 to 55 percent of patients. An increase in our knowledge concerning genetic mutations, particularly the MYD88L265P mutation, led to the development of novel targeted treatment options such as BTK inhibitors. Similarly, due to the increasing knowledge of the pathophysiology of anti-MAG polyneuropathy, new treatment options are emerging. Since anti-MAG polyneuropathy is a rare disease with diverse symptomatology, large trials with good outcome measures are a challenge. Full article
(This article belongs to the Special Issue Waldenström Macroglobulinaemia and Related Conditions)
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13 pages, 1163 KiB  
Review
Transformed Waldenström Macroglobulinemia: Update on Diagnosis, Prognosis and Treatment
by Eric Durot, Cécile Tomowiak, Elise Toussaint, Pierre Morel, Dipti Talaulikar, Prashant Kapoor, Jorge J. Castillo and Alain Delmer
Hemato 2022, 3(4), 650-662; https://doi.org/10.3390/hemato3040044 - 12 Oct 2022
Cited by 1 | Viewed by 2882
Abstract
Histological transformation (HT) to an aggressive lymphoma results from a rare evolution of Waldenström macroglobulinemia (WM). A higher incidence of transformation events has been reported in MYD88 wild-type WM patients. HT in WM can be histologically heterogeneous, although the diffuse large B-cell lymphoma [...] Read more.
Histological transformation (HT) to an aggressive lymphoma results from a rare evolution of Waldenström macroglobulinemia (WM). A higher incidence of transformation events has been reported in MYD88 wild-type WM patients. HT in WM can be histologically heterogeneous, although the diffuse large B-cell lymphoma of activated B-cell subtype is the predominant pathologic entity. The pathophysiology of HT is largely unknown. The clinical suspicion of HT is based on physical deterioration and the rapid enlargement of the lymph nodes in WM patients. Most transformed WM patients present with elevated serum lactate dehydrogenase (LDH) and extranodal disease. A histologic confirmation regarding the transformation to a higher-grade lymphoma is mandatory for the diagnosis of HT, and the choice of the biopsy site may be dictated by the findings of the 18fluorodeoxyglucose-positron emission tomography/computed tomography. The prognosis of HT in WM is unfavorable, with a significantly inferior outcome compared to WM patients without HT. A validated prognostic score based on 3 adverse risk factors (elevated LDH, platelet count < 100 × 109/L and any previous treatment for WM) stratifies patients into 3 risk groups. The most common initial treatment used is a chemo-immunotherapy (CIT), such as R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone). The response duration is short and central nervous system relapses are frequent. Whether autologous stem cell transplantation could benefit fit patients responding to CIT remains to be studied. Full article
(This article belongs to the Special Issue Waldenström Macroglobulinaemia and Related Conditions)
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16 pages, 8790 KiB  
Review
B Lymphoproliferative Neoplasms of Uncertain Biological Significance: Report from the IV Workshop of the Italian Group of Hematopathology and Review of the Literature
by Gioia Di Stefano, Francesca Magnoli, Massimo Granai, Federico Vittone, Raffaella Santi, Domenico Ferrara, Emanuela Boveri, Ada M. Florena, Falko Fend, Elena Sabattini, Marco Paulli, Maurilio Ponzoni, Stefano Lazzi, Stefano A. Pileri, Lorenzo Leoncini and the Italian Group of Hematopathology
Hemato 2022, 3(4), 634-649; https://doi.org/10.3390/hemato3040043 - 3 Oct 2022
Cited by 1 | Viewed by 2113
Abstract
Lymphoproliferative neoplasms of uncertain biological significance are increasingly encountered due to widespread usage of immunophenotypic and molecular techniques. Considering that clearer biological criteria and patient management have been established for B-cell lymphoproliferative diseases of undetermined significance occurring in the peripheral blood, many issues [...] Read more.
Lymphoproliferative neoplasms of uncertain biological significance are increasingly encountered due to widespread usage of immunophenotypic and molecular techniques. Considering that clearer biological criteria and patient management have been established for B-cell lymphoproliferative diseases of undetermined significance occurring in the peripheral blood, many issues are still obscure for early lesions detected in lymphoid tissues. Regardless that some categories of lymphoproliferative neoplasms of uncertain biological significance have been recognized by the 4th edition of the WHO, other anecdotal early lymphoproliferative lesions still remain fully undefined. Some early lesions frequently originate from the germinal center, including atypical germinal centers BCL2-negative, an early pattern of large B-cell lymphoma with IRF4 rearrangement, and “in situ” high-grade B lymphomas. Moreover, other early lymphoproliferative lesions arise outside the germinal center and include those developing within the setting of monocytoid B-cell hyperplasia, but they also can be directly or indirectly associated with chronic inflammations. This review aims to summarize the concepts discussed during the IV Workshop organized by the Italian Group of Hematopathology, focus on the state-of-the-art on B-cell lymphoproliferative neoplasms of uncertain biological significance, and offer operative insights to pathologists and clinicians in routine diagnostics. Full article
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19 pages, 928 KiB  
Review
Genetics of Transformed Follicular Lymphoma
by Miguel Alcoceba, María García-Álvarez, Jessica Okosun, Simone Ferrero, Marco Ladetto, Jude Fitzgibbon and Ramón García-Sanz
Hemato 2022, 3(4), 615-633; https://doi.org/10.3390/hemato3040042 - 1 Oct 2022
Viewed by 2864
Abstract
Histological transformation (HT) to a more aggressive disease–mostly diffuse large B-cell lymphoma–is considered one of the most dismal events in the clinical course of follicular lymphoma (FL). Current knowledge has not found a single biological event specific for HT, although different studies have [...] Read more.
Histological transformation (HT) to a more aggressive disease–mostly diffuse large B-cell lymphoma–is considered one of the most dismal events in the clinical course of follicular lymphoma (FL). Current knowledge has not found a single biological event specific for HT, although different studies have highlighted common genetic alterations, such as TP53 and CDKN2A/B loss, and MYC translocations, among others. Together, they increase genomic complexity and mutational burden at HT. A better knowledge of HT pathogenesis would presumably help to find diagnostic biomarkers allowing the identification of patients at high-risk of transformation, as well as the discrimination from patients with FL recurrence, and those who remain in remission. This would also help to identify new drug targets and the design of clinical trials for the treatment of transformation. In the present review we provide a comprehensive overview of the genetic events frequently identified in transformed FL contributing to the switch towards aggressive behaviour, and we will discuss current open questions in the field of HT. Full article
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20 pages, 2042 KiB  
Review
Molecular Pathogenesis of Follicular Lymphoma: From Genetics to Clinical Practice
by Cristina López, Pablo Mozas, Armando López-Guillermo and Sílvia Beà
Hemato 2022, 3(4), 595-614; https://doi.org/10.3390/hemato3040041 - 26 Sep 2022
Cited by 5 | Viewed by 4464
Abstract
Follicular lymphoma (FL), a generally indolent disease that derives from germinal center (GC) B cells, represents around 20–25% of all new lymphomas diagnosed in Western countries. The characteristic t(14;18)(q32;q21) translocation that places the BCL2 oncogene under control of the immunoglobulin heavy-chain enhancer occurs [...] Read more.
Follicular lymphoma (FL), a generally indolent disease that derives from germinal center (GC) B cells, represents around 20–25% of all new lymphomas diagnosed in Western countries. The characteristic t(14;18)(q32;q21) translocation that places the BCL2 oncogene under control of the immunoglobulin heavy-chain enhancer occurs in pro- or pre-B cells. However, additional secondary alterations are required for the development of overt FL, which mainly affects genes involved in epigenetic and transcriptional regulation, signaling and B cell differentiation, the BCR/NF-κB pathway, and proliferation/apoptosis. On the other hand, new insights into the FL pathogenesis suggest that FL lacking the BCL2 translocation might be a distinct biological entity with genomic features different from the classical FL. Although FL is considered an indolent disease, around 10–20% of cases eventually transform to an aggressive lymphoma, usually a diffuse large B cell lymphoma, generally by a divergent evolution process from a common altered precursor cell acquiring genomic alterations involved in the cell cycle and DNA damage responses. Importantly, FL tumor cells require interaction with the microenvironment, which sustains cell survival and proliferation. Although the use of rituximab has improved the outlook of most FL patients, further genomic studies are needed to identify those of high risk who can benefit from innovative therapies. This review provides an updated synopsis of FL, including the molecular and cellular pathogenesis, key events of transformation, and targeted treatments. Full article
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21 pages, 2770 KiB  
Review
Cold Agglutinin Disease: Improved Understanding of Pathogenesis Helps Define Targets for Therapy
by Sigbjørn Berentsen, Shirley D’Sa, Ulla Randen, Agnieszka Małecka and Josephine M. I. Vos
Hemato 2022, 3(4), 574-594; https://doi.org/10.3390/hemato3040040 - 20 Sep 2022
Cited by 6 | Viewed by 7168
Abstract
The last 2 decades have seen great progress in understanding the pathogenesis of cold agglutinin disease (CAD) and development of effective therapies. Cold agglutinins can cause hemolytic anemia as well as peripheral circulatory symptoms such as acrocyanosis. We distinguish CAD, a well-defined clinicopathologic [...] Read more.
The last 2 decades have seen great progress in understanding the pathogenesis of cold agglutinin disease (CAD) and development of effective therapies. Cold agglutinins can cause hemolytic anemia as well as peripheral circulatory symptoms such as acrocyanosis. We distinguish CAD, a well-defined clinicopathologic entity, from secondary cold agglutinin syndrome. This review addresses the histopathologic, immune phenotypic, and molecular features that allow CAD to be classified as a distinct clonal lymphoproliferative disorder of the bone marrow, recently recognized in the WHO classification. We discuss recent data on the possible overlap or distinction between CAD and Waldenström’s macroglobulinemia. Two major steps in the pathogenesis of CAD are identified: clonal B-cell lymphoproliferation (leading to monoclonal IgM production) and complement-mediated hemolysis. Each of these steps constitutes a target for treatment. Established as well as novel and experimental therapies are reviewed. Full article
(This article belongs to the Special Issue Waldenström Macroglobulinaemia and Related Conditions)
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