DOCSLIB.ORG

Cytogenetic Studies and Clinical Aspects of Patients with Plasma Cell Leukemia and Leukemic Macroglobulinemia 1

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Cytogenetic Studies and Clinical Aspects of Patients with Plasma Cell Leukemia and Leukemic Macroglobulinemia 1 [CANCER RESEARCH 43, 905-912, February 1983] 0008-5472/83/0043-0000502.00 Cytogenetic Studies and Clinical Aspects of Patients with Plasma Cell Leukemia and Leukemic Macroglobulinemia 1 Yoshimi Ueshima, 2 Shirou Fukuhara, Kenichi Nagai, Kiyoshi Takatsuki, and Haruto Uchino First Division, Department of Internal Medicine, Faculty of Medicine, Kyoto University, 54-Shogoin Kawaramachi, Sakyo-ku, Kyoto 606 [Y. U., S. F., K. N., H. U.], and Second Division, Department of Internal Medicine, Faculty of Medicine, Kumamoto University, Kumamoto [K. T.], Japan ABSTRACT evolution of myeloma cells (1 6). This assumption remains ten- tative because of lack of data in PCL. Chromosomes of six patients with plasma cell leukemia and We, rc, nnrt fh~ nhrnmns s163 ~ncl fh# r:linir~l ~ncl one patient with leukemic macroglobulinemia were examined ..... r .................................................. morphological features in 6 patients with PCL and in one from peripheral blood, bone marrow, and/or pleural fluid. All patient with leukemic MG. The karyotypic differences between the patients had a clonal chromosomal abnormality. The modal these patients and reported cases of leukemias, malignant chromosome number was near tetraploid in two, pseudodiploid lymphomas, and MM are discussed. in two, and hypodiploid in three patients. Rearrangements of chromosome 1 were found in all the patients. The most con- sistent abnormality was a large marker involving the long arm MATERIALS AND METHODS of No. 1, found in six patients, including the patient with Chromosomes of 6 patients with PCL and one patient with leukemic macroglobulinemia. Each patient had one to four large markers MG were examined from peripheral blood, bone marrow, and/or which resulted in partial trisomy to hexasomy for the long arm pleural effusion specimens. PCL was defined as more than 0.5 x 109 of No. 1. The translocations occurred with No. 9 in two, with circulating plasma cells or plasmoblasts per liter. The peripheral blood No. 1 6 in two, and Nos. 8, 1 7, and 1 8 each in one patient. The mononuclear cells were separated with Ficoll-sodium metrizoate gra- survival time from the diagnosis was less than 1 year in five of dient centrifugation and were cultured in Roswell Park Memorial Insti- them and over 2 years in one. The only patient whose cells tute Medium 1640 containing 10% fetal calf serum at 37 ~ for 24 to 48 lacked an extra lq lived for over 3 years. Five 14q+ marker hr in 5% CO2. Bone marrow and pleural fluid samples were processed directly without prior culture. Mitotic cells were arrested with 1 x 10 -6 chromosomes were detected in three patients. The donor chro- M colchicine for 2 hr, treated with 0.075 M KCI for 10 to 30 min, and mosome was No. 1 1 in one of these and was undetermined in subsequently fixed in methanol:acetic acid (3:1). Air-dried slides were the others; the size of each 14q+ marker seemed quite differ- first stained with Giemsa and then photographed; after destaining, the ent which suggested different donor chromosomes. Loss of a same cells were restained with quinacrine mustard and photographed sex chromosome was found in five patients. Loss of No. 13 with a fluorescence microscope. Case 1 was banded with a G-banding and gain of No. 7 or 7q were each found in two patients. method (21). Descriptions of the chromosomes follow the recommen- Rearrangement or deletion of the short arm of No. 8 was found dations of the ISCN (1978) (9). in five patients. A rearrangement of 9p was found in three patients. The myeloma cells had a different morphology in the peripheral blood, bone marrow, and/or pleural fluid before and RESULTS after the leukemic phase of one patient; however, chromosome Clinical data on the 7 patients are presented in Table 1. Four analysis revealed the same clone despite the altered morphol- patients had PCL at the time of initial diagnosis, and in 2 ogy. patients (Cases 1 and 5) a leukemic phase subsequently de- veloped. The patient with MG (Case 7) also had leukemic INTRODUCTION plasmacytoid cells at diagnosis. The survival after diagnosis was less than 1 year in 5 patients and over 2 years in 2. All the PCL 3 is an infrequent phase in MM. The clinical course may patients had anemia, hypercalcemia, and renal insufficiency. be fulminant with features resembling those of acute leukemia, Three had hepatosplenomegaly (Cases 1, 2, and 5), and 3 and it is sometimes recognized as a distinct subentity (28). (Cases 2, 5, and 6) had pleural effusion with plasma cell Chromosome studies in patients with MM and related parapro- invasion. In addition, one had progressive lower limb paresis teinemic disorders have often been reported (1,3, 1 0, 1 1, 1 5, (Case 2), one had meningeal involvement (Case 5), and another 16, 22, 23, 26, 29), but there are few reports of PCL (8, 1 1, had obstructive jaundice with plasma cell invasion (Case 6). 16, 26). In MM, the pattern of chromosome aberrations has Postmortem examination in 3 patients (Cases 1, 2, and 5) b,~en quite variable, and no consistent markers were noted in showed very extensive infiltration of the marrow, liver, lymph the published data. On the other hand, it has been suggested nodes, lungs, and other organs with plasma cells. that the 14q+ marker may be necessary for the leukemic Cytogenetic data are presented in Table 2. Chromosomes were studied before treatment in 2 patients, after treatment in Supported in part by a grant from the Ministry of Welfare of Japan. 4 patients, and before and after treatment in one patient. There 2 To whom requests for reprints should be addressed, at M.D. Department of were no clear differences between the karyotypes of untreated Internal Medicine, Faculty of Medicine, Kyoto University, 54-Shogoin Kawara- machi, Sakyoku, Kyoto 606, Japan. Present address: M.D. Department of Med- patients and those of treated patients. All of the patients had a icine, Box 420, University of Chicago, Chicago, II1. 60637. clonal chromosomal abnormality. In 2 patients (Cases 1 and The abbreviations used are: PCL, plasma cell leukemia; MM, multiple mye- Ioma; MG, macroglobulinemia. 2), the cells from bone marrow, peripheral blood, and/or Received June 17, 1982; accepted October 11, 1982. pleural effusion had the same karyotype. In another patient FEBRUARY1983 905 Downloaded from cancerres.aacrjournals.org on September 24, 2021. © 1983 American Association for Cancer Research. Y. Ueshima et al. Table 1 Clinical and hematological data in 6 patients with PCL and a patient with MG at diagnosis of PCL % of plasma cells WBC in pe- and atypical lym- Therapy before Survival after Age Serum para- ripheral blood phocytes in periph- chromosome ex- Total duration of chromosome ex- Case a Sex (yr) protein (x 109/liter) eral blood amination disease (mos.) amination (mos.) 1 (M. Y.) M 45 Absent 52.1 90 M, b P 39 2 2 (Y. N.) M 35 IgG~ 7.6 33 E, P 10 7 3 (S. I.) F 71 IgG• 9.7 72 None 1 1 4 iT. Y.) M 73 BJ~ 20.7 10 None 1.5 1.5 5 (N. Y.) M 45 IgGX 8.2 37 (1) None 8 (1) 8 (2) M, P (2) 4 (3) 0 6 (S. K.) F 64 IgAK 6.4 22 M, P 34 (1),31 (2) 2 7 (M. H.) M 71 IgMX 78.0 90. D, C, 6-MP, P 12 8 a Cases 1 to 6, PCL; Case 7, MG. b M, melphalan; P, prednisone; E, endoxan; D, daunomycin; C, 1-/~-D-arabinoside furanosylcytosine; 6-MP, 6-mercaptopurine. Table 2 Chromosome patterns of patients with PCL and MG Date of chro- No. of cells ex- % of cells with Source of mosome stud- amined abnormal kary- Patient sample ies (banded) otype Modal karyotype 1 (M. Y.) BM a 9/21/77 20 100 44,X,-Y,-21,-22,1p-,+1q-,t(11 ;14)(ql 1 ;q32),14q+ PB 10/15/77 19 1 O0 44,X,-Y,-21,-22,1 p-, + 1q-,t(11 ;14)(ql 1 ;q32), 14q+ 2 (Y. N.) BM 4/12/79 16 100 44,X,-Y,-9,- 13,t(6;8)(q 13;pl 1 ), + der(9)t(1 ;9)(1 qter--> 1 ql 2::1 q44--* 1 ql 2: :9q34--> 9pter) PB 4/12/79 9 100 44,X,-Y,-9,- 13,t(6;8)(q 13;pl 1 ), + der(9)t(1 ;9) PE 4/12/79 7 86 44,X,-Y,-9,-13,t(6;8)(q 13;pl 1 ), + der(9)t(1 ;9) 3 (S. I.) PB 10/25/79 40 20 43,X,-X,- 1,-8,-9,- 1 O,- 13,- 22, + der(8)t(1 ;8)(1 qter---*cen--,8qter), +der(9)t(1 ;9)(1 qter--* 1q21 ::9p24--*9qter), + der(10)t(1 ;10) (10pter-* 10q26:: 1 p31--* 1 pter), + t(1 ; ?)(q 12;?) 4(T. Y.) PB 12/26/79 38 76 78,XX,-Y, +4,+5,+7,+ 7,+ 7,+7, +8, +8,+ 10,+ 12, + 12, + 16,+ 18,+ 19,+ 19,+21, +21,+3q+,+3q+,+3p-,+9p+,+9p+,+11p+,+11p+,+14q+,+14q+, + 1 6p +, + 16p+, + del(1 )(q21 ), + del(1 )(q21 ), + der(17)t(1 ; 17) (1 qter--->1 ql 2::17pl 3-,17qter), + der(17)t(1 ;17) 5 (N. u BM 1/12/80 14 85 46,XY,- 16,- 18,del(6)(q21 ), + der(16)t(1 ;16)(1 qter-* 1 q21 :: 16pl 3--->16qter), + marl PB 5/21/80 16 100 46,XY, - 16, - 18,del(6)(q21 ), + der(16)t(1 ;16), + marl PE 9/06/80 20 100 47,XY,-8,- 15,- 16,- 18,del(6)(q21 ), + der(16)t(1 ;16), + t(8; 15)(8qter--*8p23::15ql 2 --,15qter),14q+,+mar1 ,+mar2,+mar3/sdl (4 cells)47,XY, same, 4q+ 6(S.
Load more

Recommended publications
  • MYD88 L265P Is a Marker Highly Characteristic Of, but Not Restricted To, Waldenstro¨M’S Macroglobulinemia
    Leukemia (2013) 27, 1722–1728 & 2013 Macmillan Publishers Limited All rights reserved 0887-6924/13 www.nature.com/leu ORIGINAL ARTICLE MYD88 L265P is a marker highly characteristic of, but not restricted to, Waldenstro¨m’s macroglobulinemia C Jime´ nez1, E Sebastia´n1, MC Chillo´n1,2, P Giraldo3, J Mariano Herna´ndez4, F Escalante5, TJ Gonza´lez-Lo´ pez6, C Aguilera7, AG de Coca8, I Murillo3, M Alcoceba1, A Balanzategui1, ME Sarasquete1,2, R Corral1, LA Marı´n1, B Paiva1,2, EM Ocio1,2, NC Gutie´ rrez1,2, M Gonza´lez1,2, JF San Miguel1,2 and R Garcı´a-Sanz1,2 We evaluated the MYD88 L265P mutation in Waldenstro¨m’s macroglobulinemia (WM) and B-cell lymphoproliferative disorders by specific polymerase chain reaction (PCR) (sensitivity B10 À 3). No mutation was seen in normal donors, while it was present in 101/117 (86%) WM patients, 27/31 (87%) IgM monoclonal gammapathies of uncertain significance (MGUS), 3/14 (21%) splenic marginal zone lymphomas and 9/48 (19%) non-germinal center (GC) diffuse large B-cell lymphomas (DLBCLs). The mutation was absent in all 28 GC-DLBCLs, 13 DLBCLs not subclassified, 35 hairy cell leukemias, 39 chronic lymphocyticleukemias(16withM-component), 25 IgA or IgG-MGUS, 24 multiple myeloma (3 with an IgM isotype), 6 amyloidosis, 9 lymphoplasmacytic lymphomas and 1 IgM-related neuropathy. Among WM and IgM- MGUS, MYD88 L265P mutation was associated with some differences in clinical and biological characteristics, although usually minor; wild-type MYD88 cases had smaller M-component (1.77 vs 2.72 g/dl, P ¼ 0.022), more lymphocytosis (24 vs 5%, P ¼ 0.006), higher lactate dehydrogenase level (371 vs 265 UI/L, P ¼ 0.002), atypical immunophenotype (CD23 À CD27 þþFMC7 þþ), less Immunoglobulin Heavy Chain Variable gene (IGHV) somatic hypermutation (57 vs 97%, P ¼ 0.012) and less IGHV3–23 gene selection (9 vs 27%, P ¼ 0.014).
    [Show full text]
  • Spotlight on Ixazomib: Potential in the Treatment of Multiple Myeloma Barbara Muz Washington University School of Medicine in St
    Washington University School of Medicine Digital Commons@Becker Open Access Publications 2016 Spotlight on ixazomib: Potential in the treatment of multiple myeloma Barbara Muz Washington University School of Medicine in St. Louis Rachel N. Ghazarian Washington University School of Medicine in St. Louis Monica Ou Washington University School of Medicine in St. Louis Micha J. Luderer Washington University School of Medicine in St. Louis Hubert D. Kusdono Washington University School of Medicine in St. Louis See next page for additional authors Follow this and additional works at: https://digitalcommons.wustl.edu/open_access_pubs Recommended Citation Muz, Barbara; Ghazarian, Rachel N.; Ou, Monica; Luderer, Micha J.; Kusdono, Hubert D.; and Azab, Abdel K., ,"Spotlight on ixazomib: Potential in the treatment of multiple myeloma." Drug Design, Development and Therapy.2016,10. 217-226. (2016). https://digitalcommons.wustl.edu/open_access_pubs/5207 This Open Access Publication is brought to you for free and open access by Digital Commons@Becker. It has been accepted for inclusion in Open Access Publications by an authorized administrator of Digital Commons@Becker. For more information, please contact [email protected]. Authors Barbara Muz, Rachel N. Ghazarian, Monica Ou, Micha J. Luderer, Hubert D. Kusdono, and Abdel K. Azab This open access publication is available at Digital Commons@Becker: https://digitalcommons.wustl.edu/open_access_pubs/5207 Journal name: Drug Design, Development and Therapy Article Designation: Review Year: 2016 Volume:
    [Show full text]
  • Initial Evaluation of the Patient with Waldenstro¨ M Macroglobulinemia Can Be Challenging
    Initial Evaluation of the Patient with Waldenstro¨m Macroglobulinemia Jorge J. Castillo, MD*, Steven P. Treon, MD, PhD KEYWORDS Waldenstro¨ m macroglobulinemia Bone marrow aspiration Anemia Hyperviscosity Cryoglobulinemia Peripheral neuropathy Bing-Neel syndrome Amyloidosis KEY POINTS The initial evaluation of the patient with Waldenstro¨ m macroglobulinemia can be challenging. Not only is Waldenstro¨ m macroglobulinemia a rare disease, but the clinical features of pa- tients with Waldenstro¨ m macroglobulinemia can vary greatly from patient to patient. The authors provide concise and practical recommendations for the initial evaluation of patients with Waldenstro¨ m macroglobulinemia, specifically regarding history taking, physical examination, laboratory testing, bone marrow aspiration and biopsy evaluation, and imaging studies. The authors review the most common special clinical situations seen in patients with Wal- denstro¨ m macroglobulinemia, especially anemia, hyperviscosity, cryoglobulinemia, pe- ripheral neuropathy, extramedullary disease, Bing-Neel syndrome, and amyloidosis. INTRODUCTION Given its rarity and a highly variable clinical presentation, the initial evaluation of the patient with a clinicopathologic diagnosis of Waldenstro¨ m macroglobulinemia (WM) can be challenging. The clinical manifestations of WM can be associated with infiltra- tion of the bone marrow and other organs by malignant lymphoplasmacytic cells and/ or the properties of the monoclonal IgM paraproteinemia, and include anemia, hyper- viscosity,
    [Show full text]
  • Understanding the Cryoglobulinemias
    Current Rheumatology Reports (2019) 21:60 https://doi.org/10.1007/s11926-019-0859-0 VASCULITIS (L ESPINOZA, SECTION EDITOR) Understanding the Cryoglobulinemias Alejandro Fuentes1 & Claudia Mardones1 & Paula I. Burgos1 # Springer Science+Business Media, LLC, part of Springer Nature 2019 Abstract Purpose of the Review Cryoglobulins are immunoglobulins with the ability to precipitate at temperatures <37 °C. They are related to hematological disorders, infections [especially hepatitis C virus (HCV)], and autoimmune diseases. In this article, the state of the art on Cryoglobulinemic Vasculitis (CV), in a helpful and schematic way, with a special focus on HCV related Mixed Cryoglobulinemia treatment are reviewed. Recent Findings Direct – acting antivirals (DAA) against HCV have emerged as an important key in HCV treatment to related Cryoglobulinemic Vasculitis, and should be kept in mind as the initial treatment in non–severe manifestations. On the other hand, a recent consensus panel has published their recommendations for treatment in severe and life threatening manifestations of Mixed Cryoglobulinemias. Summary HCV-Cryoglobulinemic vasculitis is the most frequent form of CV. There are new treatment options in HCV-CV with DAA, with an important number of patients achieving complete response and sustained virologic response (SVR). In cases of severe forms of CV, treatment with Rituximab and PLEX are options. The lack of data on maintenance therapy could impulse future studies in this setting. Keywords HCV . Mixed Cryoglobulinemia . Type I Cryoglobulinemia . gC1qR . Direct-acting antivirals . Rituximab Introduction and Definitions tion of the total pool of cryoprecipitable immunocomplexes in targeted vessels and due to false negative results owing to im- Cryoglobulins are immunoglobulins (Ig) that precipitate in vitro proper blood sampling or inadequate laboratory processes [4].
    [Show full text]
  • Waldenstrom's Macroglobulinemia
    Review Article Open Acc Blood Res Trans J Volume 3 Issue 1 - May 2019 Copyright © All rights are reserved by Mostafa Fahmy Fouad Tawfeq DOI: 10.19080/OABTJ.2019.02.555603 Waldenstrom’s Macroglobulinemia: An In-depth Review Sabry A Allah Shoeib1, Essam Abd El Mohsen2, Mohamed A Abdelhafez1, Heba Y Elkholy1 and Mostafa F Fouad3* 1Internal Medicine Department , Faculty of Medicine, Menoufia University 2El Maadi Armed Forces Institute, Egypt 3Specialist of Internal Medicine at Qeft Teatching Hospital, Qena, Egypt Submission: March 14, 2019; Published: May 20, 2019 *Corresponding author: Mostafa Fahmy Fouad Tawfeq MBBCh, Adress: Elzaferia, Qeft, Qena, Egypt Abstract Objective: The aim of the work was to through in-depth lights on new updates in waldenstrom macroglobulinemia disease. Data sources: Data were obtained from medical textbooks, medical journals, and medical websites, which had updated with the key word (waldenstrom macroglobulinemia ) in the title of the papers. Study selection: Selection was carried out by supervisors for studying waldenstrom macroglobulinemia disease. Data extraction: Special search was carried out for the key word waldenstrom macroglobulinemia in the title of the papers, and extraction was made, including assessment of quality and validity of papers that met with the prior criteria described in the review. Data synthesis: The main result of the review and each study was reviewed independently. The obtained data were translated into a new language based on the need of the researcher and have been presented in various sections throughout the article. Recent Findings: We now know every updated information about Wald Enstrom macroglobulinemia and clinical trials. A complete understanding of the Wald Enstrom macroglobulinemia will be helpful for the future development of innovative therapies for the treatment of the disease and its complications.
    [Show full text]
  • Primary Plasma Cell Leukemia: a Practical Approach to Diagnosis and Clinical Management
    PRIMARY PLASMA CELL LEUKEMIA: A PRACTICAL APPROACH TO DIAGNOSIS AND CLINICAL MANAGEMENT Primary Plasma Cell Leukemia: A Practical Approach to Diagnosis and Clinical Management Wilson I. Gonsalves, MD Abstract clonal plasma cells. Although Gluzinski and Reichenstein were the 1 Primary plasma cell leukemia (pPCL) represents a rare first to describe a case of PCL back in 1906, it was Kyle and Noel but most aggressive form of multiple myeloma. Its who went on to define it as the presence of plasma cells consisting leukemic clinical characteristics, as seen by the presence of more than 20% of the differential white count in the peripheral of circulating clonal plasma cells, its unique molecular blood, or an absolute plasma cell peripheral blood count of greater 9 2 and cytogenetic aberrations, and its exceedingly poor than 2.0 x 10 cells/L. If PCL is detected at diagnosis de novo survival outcomes set it apart from traditional multiple without a prior history of MM, it is considered primary plasma cell myeloma. Recent advances in the utilization of novel leukemia (pPCL). HoWever, if PCL arises in a patient with a known agents and high-dose chemotherapy in the upfront man- history of MM, it is considered secondary PCL (sPCL). The con- agement of patients with pPCL is finally bearing fruit in dition occurs as a progressive event of the disease in 1% to 4% of 3 terms of improving survival outcomes, albeit modestly. patients with MM. With the improvement in survival experienced 4 Early recognition of pPCL in a newly diagnosed multiple by patients with MM, many are living long enough to have their myeloma patient is crucial for providing the optimal MM transform into sPCL.
    [Show full text]
  • Progression of a Solitary, Malignant Cutaneous Plasma-Cell Tumour to Multiple Myeloma in a Cat
    Case Report Progression of a solitary, malignant cutaneous plasma-cell tumour to multiple myeloma in a cat A. Radhakrishnan1, R. E. Risbon1, R. T. Patel1, B. Ruiz2 and C. A. Clifford3 1 Mathew J. Ryan Veterinary Hospital of the University of Pennsylvania, Philadelphia, PA, USA 2 Antech Diagnostics, Farmingdale, NY, USA 3 Red Bank Veterinary Hospital, Red Bank, NJ, USA Abstract An 11-year-old male domestic shorthair cat was examined because of a soft-tissue mass on the left tarsus previously diagnosed as a malignant extramedullary plasmacytoma. Findings of further diagnostic tests carried out to evaluate the patient for multiple myeloma were negative. Five Keywords hyperproteinaemia, months later, the cat developed clinical evidence of multiple myeloma based on positive Bence monoclonal gammopathy, Jones proteinuria, monoclonal gammopathy and circulating atypical plasma cells. This case multiple myeloma, pancytopenia, represents an unusual presentation for this disease and documents progression of an plasmacytoma extramedullary plasmacytoma to multiple myeloma in the cat. Introduction naemia, although it also can occur with IgG or IgA Plasma-cell neoplasms are rare in companion ani- hypersecretion (Matus & Leifer, 1985; Dorfman & mals. They represent less than 1% of all tumours in Dimski, 1992). Clinical signs of hyperviscosity dogs and are even less common in cats (Weber & include coagulopathy, neurologic signs (dementia Tebeau, 1998). Diseases represented in this category and ataxia), dilated retinal vessels, retinal haemor- of neoplasia include multiple myeloma (MM), rhage or detachment, and cardiomyopathy immunoglobulin M (IgM) macroglobulinaemia (Dorfman & Dimski, 1992; Forrester et al., 1992). and solitary plasmacytoma (Vail, 2001). These con- Coagulopathy can result from the M-component ditions can result in an excess secretion of Igs interfering with the normal function of platelets or (paraproteins or M-component) which produce a clotting factors.
    [Show full text]
  • A Case of Rare Subtype of Multiple Myeloma: Secondary Plasma Cell Leukemia Author Affiliations Are Listed at the End of This Article
    Sanwal et al. HCA Healthcare Journal of Medicine (2021) 2:1 https://doi.org/10.36518/2689-0216.1114 Case Report A Case of Rare Subtype of Multiple Myeloma: Secondary Plasma Cell Leukemia Author affiliations are listed at the end of this article. Chandra Sanwal, DO,1 Aftab Mahmood, MD,1 Michael Bailey, MD,2 Krutika Patel, MD,1 Antonio Guzman, MD1 Correspondence to: Chandra Sanwal, DO Abstract Corpus Christi Medical Description Center Plasma cell leukemia is a rare, aggressive form of multiple myeloma with the presence of 7101 S Padre Island Dr circulating plasma cells in the peripheral blood. There are two types of plasma cell leukemia, Corpus Christi, TX 78412 primary and secondary, depending on if there was previous evidence of multiple myeloma. The diagnostic criterion of plasma cell leukemia is based on a percentage (>20%) or an abso- (chandrasanwal7@gmail. lute number of (≥2 x 109/L) plasma cells in the peripheral circulation. We present the clinical com) course of a rare case of secondary plasma cell leukemia in a patient from the time of initial diagnosis of multiple myeloma, its remission period of about 5 years, and its final progres- sion into refractory secondary plasma cell leukemia. This case report details the patient’s presenting symptoms, pertinent laboratory and diagnostic imaging findings, and histopa- thology of peripheral blood and bone marrow. This case report presents a chronological comparison of key laboratory findings that manifest the progression of multiple myeloma into secondary plasma cell leukemia. It also offers a brief review of the literature for the diagnosis and treatment of plasma cell leukemia.
    [Show full text]
  • Significance of Antiphospholipid Antibodies in Essential Cryoglobulin- Emia: a Case Report and Review of Literature Rama Atluri and Mian Muhammad Rizwan*
    Atluri and Rizwan. Clin Med Rev Case Rep 2017, 4:151 Volume 4 | Issue 1 Clinical Medical Reviews ISSN: 2378-3656 and Case Reports Case Report: Open Access Significance of Antiphospholipid Antibodies in Essential Cryoglobulin- emia: A Case Report and Review of Literature Rama Atluri and Mian Muhammad Rizwan* Division of Rheumatology, Saint Louis University, St Louis, USA *Corresponding author: Mian Muhammad Rizwan, Fellow Rheumatology, Division of Rheumatology, 1402 South Grand Boulevard, Doisy Hall, Room 203, St Louis, MO 63104, USA, Tel: 314-977-8832, Fax: 314-977-8818, E-mail: [email protected] monoclonal cryoglobulins. In 1990s it was established that most Abstract of these essential MC are associated with chronic hepatitis C virus Cryoglobulinemia is a rare immune-complex-mediated small vessel (HCV) infection [2,3]. We now know that MC is associated with vasculitis that has a smoldering clinical course and can potentially clinical situations that generate large amounts of IgG and immune involve multiple organ systems. The discovery of its relationship complexes, including chronic autoimmune diseases such as systemic with hepatitis C infection shows the striking association between lupus erythematosus, Sjögren’s syndrome [4] or infections such as a viral infection, an autoimmune disease and lymphoproliferative HCV and HIV infections. MC not associated with those disorders disorders. It is estimated that hepatitis C negative cryoglobulinemia accounts for about 5% to 10% of cases. There have been sporadic has been defined as essential (primary) MC. The clinical features, reports of association between cryoglobulins and antiphospholipid etiologies, and treatment of MC not associated with HCV infection antibody leading to the suspicion that they participate in the have been poorly described.
    [Show full text]
  • Plasma Cell Leukemia – Facts and Controversies: More Questions Than Answers?
    Clinical Hematology International Vol. 2(4); December (2020), pp. 133–142 DOI: https://doi.org/10.2991/chi.k.200706.002; eISSN 2590-0048 https://www.atlantis-press.com/journals/chi/ Review Plasma Cell Leukemia – Facts and Controversies: More Questions than Answers? Anna Suska1, David H. Vesole2, Jorge J. Castillo3, Shaji K. Kumar4, Hari Parameswaran5, Maria V. Mateos6, Thierry Facon7, Alessandro Gozzetti8, Gabor Mikala9, Marta Szostek1, Joseph Mikhael10, Roman Hajek11, Evangelos Terpos12, Artur Jurczyszyn1,* 1Department of Hematology, Jagiellonian University Medical College, Kopernika 17, Krakow 31-501, Poland 2The John Theurer Cancer Center at Hackensack UMC, Hackensack, NJ, USA 3Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA 4Division of Hematology, Mayo Clinic, Rochester, MN, USA 5Medical College of Wisconsin, Milwaukee, WI, USA 6Complejo Asistencial Universitario de Salamanca, Instituto de Investigación Biomédica de Salamanca (CAUSA/IBSAL), Salamanca, Spain 7Service des Maladies du Sang, Hôpital Claude Huriez, Lille, France 8Division of Hematology and Transplants, University of Siena, Siena, Italy 9Department of Hematology and Stem Cell Transplantation, South-Pest Central Hospital, Natl. Inst. Hematol. Infectol, Budapest, Hungary 10Translational Genomics Research Institute, City of Hope Cancer Center, Phoenix, Arizona, USA 11University Hospital Ostrava and Faculty of Medicine, University of Ostrava, Ostrava, Czech Republic 12Department of Clinical Therapeutics, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece ARTICLE INFO ABSTRACT Article History Plasma cell leukemia (PCL) is an aggressive hematological malignancy characterized by an uncontrolled clonal proliferation of Received 07 April 2020 plasma cells (PCs) in the bone marrow and peripheral blood. PCL has been defined by an absolute number of circulating PCs Accepted 01 June 2020 exceeding 2.0 × 109/L and/or >20% PCs in the total leucocyte count.
    [Show full text]
  • Ninlaro® (Ixazomib)
    Ninlaro® (ixazomib) (Oral) Document Number: IC-0261 Last Review Date: 10/26/2020 Date of Origin: 12/04/2015 Dates Reviewed: 12/04/2015, 10/2016, 10/2017, 10/2018, 11/2019, 11/2020 I. Length of Authorization 6,7 Coverage will be provided for six months and may be renewed unless otherwise specified. Waldenström Macroglobulinemia: Initial coverage will be provided for 6 months consisting of six 4-week cycles (6 doses) and may be renewed up to a maximum of six 8-week cycles (6 doses) Systemic Light Amyloidosis: Coverage may be renewed up to a maximum of twelve 4-week cycles (12 doses) II. Dosing Limits A. Quantity Limit (max daily dose) [NDC Unit]: 2.3 mg capsule: 3 capsules per 28 days 3 mg capsule: 3 capsules per 28 days 4 mg capsule: 3 capsules per 28 days B. Max Units (per dose and over time) [HCPCS Unit]: 12 mg per 28 days III. Initial Approval Criteria 1 Coverage is provided in the following conditions: Patient is at least 18 years of age; AND Universal Criteria 1 Patient will avoid concomitant use with strong CYP3A inducers (e.g., rifampin, phenytoin, carbamazepine, St. John’s Wort, etc.); AND Multiple Myeloma† Ф 1-3 Used as initial therapy; AND Proprietary & Confidential © 2020 Magellan Health, Inc. o Used in combination with lenalidomide and dexamethasone in patients who are not transplant candidates; OR o Used in combination with cyclophosphamide and dexamethasone in patients who are transplant candidates; OR Used as maintenance therapy; AND o Used as single agent therapy in patients who are transplant candidates; AND .
    [Show full text]
  • An Unfortunate Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Change (POEMS)
    Open Access Case Report DOI: 10.7759/cureus.1086 An Unfortunate Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Change (POEMS) Faraz Afridi 1 , Jorge Otoya 2 , Samantha F. Bunting 3 , Gerard Chaaya 1 1. Internal Medicine, University of Central Florida College of Medicine 2. Hematology and Medical Oncology, Osceola Regional Medical Center 3. University of Central Florida College of Medicine Corresponding author: Faraz Afridi, [email protected] Abstract POEMS syndrome is an acronym for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes, which is a rare paraneoplastic disease of monoclonal plasma cells. A mandatory criterion to diagnose POEMS syndrome is the presence of a monoclonal plasma cell dyscrasia in which plasma cell leukemia is the most aggressive form. Early identification of the features of the POEMS syndrome is critical for patients to identify an underlying plasma cell dyscrasias and to reduce the morbidity and mortality of the disease by providing early therapy. We present a case of a 64-year-old male who presented with non-specific symptoms and was found to have primary plasma cell leukemia, which was part of his unfortunate POEMS syndrome. Categories: Endocrinology/Diabetes/Metabolism, Internal Medicine, Oncology Keywords: poems syndrome, plasma cell leukemia Introduction Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome, also known as Crow-Fukase syndrome is a rare paraneoplastic disease of monoclonal plasma cells which was first reported in 1956 [1]. The acronym of “POEMS” was derived in 1980 by Bardwick and colleagues on the basis of the five unique characteristic features [2]. The POEMS syndrome can present with plasma cell dyscrasias and therefore early identification of this syndrome may aid in the clinical course of plasma cell leukemias and other plasma cell proliferative disorders.
    [Show full text]