Primary Plasma Cell Leukemia Presenting As Heart Failure: a Case
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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: -
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. -
Signs and Symptoms
Signs and symptoms For the most part, symptoms are related to disturbed bowel functions. Pain first, vomiting next and fever last has been described as classic presentation of acute appendicitis. Pain starts mid abdomen, and except in children below 3 years, tends to localize in right iliac fossa in a few hours. This pain can be elicited through various signs. Signs include localized findings in the right iliac fossa. The abdominal wall becomes very sensitive to gentle pressure (palpation). Also, there is severe pain on suddenly releasing a deep pressure in lower abdomen (rebound tenderness). In case of a retrocecal appendix, however, even deep pressure in the right lower quadrant may fail to elicit tenderness (silent appendix), the reason being that the cecum, distended with gas, prevents the pressure exerted by the palpating hand from reaching the inflamed appendix. Similarly, if the appendix lies entirely within the pelvis, there is usually complete absence of the abdominal rigidity. In such cases, a digital rectal examination elicits tenderness in the rectovesical pouch. Coughing causes point tenderness in this area (McBurney's point) and this is the least painful way to localize the inflamed appendix. If the abdomen on palpation is also involuntarily guarded (rigid), there should be a strong suspicion of peritonitis requiring urgent surgical intervention. Rovsing's sign Continuous deep palpation starting from the left iliac fossa upwards (anti clockwise along the colon) may cause pain in the right iliac fossa, by pushing bowel contents towards the ileocaecal valve and thus increasing pressure around the appendix. This is the Rovsing's sign.[5] Psoas sign Psoas sign or "Obraztsova's sign" is right lower-quadrant pain that is produced with either the passive extension of the patient's right hip (patient lying on left side, with knee in flexion) or by the patient's active flexion of the right hip while supine. -
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. -
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. -
Abdominal Examination Positioning
ABDOMINAL EXAMINATION POSITIONING Patients hands remain on his/hers side Legs, straight Head resting on pillow – if neck is flexed, ABD muscles will tense and therefore harder to palpate ABD . INSPECTION AUSCULATION PALPATION PERCUSSION INSPECTION INSPECTION Shape Skin Abnormalities Masses Scars (Previous op's - laproscopy) Signs of Trauma Jaundice Caput Medusae (portal H-T) Ascities (bulging flanks) Spider Navi-Pregnant women Cushings (red-violet) ... Hands + Mouth Clubbing Palmer Erythmea Mouth ulceration Breath (foeter ex ore) ... AUSCULTATION Use stethoscope to listen to all areas Detection of Bowel sounds (Peristalsis/Silent?? = Ileus) If no bowel sounds heard – continue to auscultate up to 3mins in the different areas to determine the absence of bowel sounds Auscultate for BRUITS!!! - Swishing (pathological) sounds over the arteries (eg. Abdominal Aorta) ... PALPATION ALWAYS ASK IF PAIN IS PRESENT BEFORE PALPATING!!! Firstly: Superficial palpation Secondly: Deep where no pain is present. (deep organs) Assessing Muscle Tone: - Guarding = muscles contract when pressure is applied - Ridigity = inidicates peritoneal inflamation - Rebound = Releasing of pressure causing pain ....... MURPHY'S SIGN Indication: - pain in U.R.Quadrant Determines: - cholecystitis (inflam. of gall bladder) - Courvoisier's law – palpable gall bladder, yet painless - cholangitis (inflam. Of bile ducts) ... METHOD Ask patient to breathe out. Gently place your hand below the costal margin on the right side at the mid-clavicular line (location of the gallbladder). Instruct to breathe in. Normally, during inspiration, the abdominal contents are pushed downward as the diaphragm moves down. If the patient stops breathing in (as the gallbladder comes in contact with the examiner's fingers) the patient feels pain with a 'catch' in breath. -
General Medicine - Surgery IV Year
1 General Medicine - Surgery IV year 1. Overal mortality rate in case of acute ESR – 24 mm/hr. Temperature 37,4˚C. Make appendicitis is: the diagnosis? A. 10-20%; A. Appendicular colic; B. 5-10%; B. Appendicular hydrops; C. 0,2-0,8%; C. Appendicular infiltration; D. 1-5%; D. Appendicular abscess; E. 25%. E. Peritonitis. 2. Name the destructive form of appendicitis. 7. A 34-year-old female patient suffered from A. Appendicular colic; abdominal pain week ago; no other B. Superficial; gastrointestinal problems were noted. On C. Appendix hydrops; clinical examination, a mass of about 6 cm D. Phlegmonous; was palpable in the right lower quadrant, E. Catarrhal appendicitis. appeared hard, not reducible and fixed to the parietal muscle. CBC: leucocyts – 3. Koher sign is: 7,5*109/l, ESR – 24 mm/hr. Temperature A. Migration of the pain from the 37,4˚C. Triple antibiotic therapy with epigastrium to the right lower cefotaxime, amikacin and tinidazole was quadrant; very effective. After 10 days no mass in B. Pain in the right lower quadrant; abdominal cavity was palpated. What time C. One time vomiting; term is optimal to perform appendectomy? D. Pain in the right upper quadrant; A. 1 week; E. Pain in the epigastrium. B. 2 weeks; C. 3 month; 4. In cases of appendicular infiltration is D. 1 year; indicated: E. 2 years. A. Laparoscopic appendectomy; B. Concervative treatment; 8. What instrumental method of examination C. Open appendectomy; is the most efficient in case of portal D. Draining; pyelophlebitis? E. Laparotomy. A. Plain abdominal film; B. -
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. -
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 . -
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. -
Abdominal Examination
ABDOMINAL EXAMINATION Dr. Ahmed Al Sarkhy Associate professor of paediatrics , KSUMC, KSU REMEMBER BEFORE STARTING... ALWAYS Introduce your self Take permission Wash your hands GASTROINTESTINAL EXAMINATION VS. ABDOMINAL EXAM General examination General inspection (ABCDE) VS Abdominal examination Growth parameters Inspection Hands and arms Palpation Face, eyes and mouth Percussion Neck Auscultation Lower limbs GENERAL INSPECTION (ABCDE) A: APPEARANCE; well, ill, irritable, toxic B; BODY BUILT: (weight, height, waist circumference) BREATHING: resp. Distress, grunting, wheezing C: COLOUR: pale, jaundice, cyanosis D: DEHYDRATION/DYSMORPHIC FEATHURES E: EXTENSIONS: Ox tubes, IV lines, cardiac monitors Signs of dehydration HANDS Nails Clubbing Koilonychia Leuconychia Palmar erythema Dupuytren’s contractures Hepatic flap HANDS Palmar erythema Dupuytren’s contractures ARMS Spider naevi (telangiectatic lesions) Bruising Wasting Scratch marks (chronic cholestasis) FACE, EYES … Conjuctival pallor (anaemia) Sclera: jaundice Cornea: Kaiser Fleischer’s rings (Wilson’s disease) Xanthelasma (chronic cholestasis) MOUTH Breath (fetor hepaticus, DKA) Lips Angular stomatitis Cheilitis Ulceration Peutz-Jeghers syndrome Gums Gingivitis, bleeding Candida albicans Pigmentation Tongue Atrophic glossitis (B12, FA def) Furring NECK AND CHEST Cervical lymphadenopathy Left supraclavicular fossa (Virchov’s node=lymphoma) Gynaecomastia Spider nevi NLOWER LIMBS VASCULAITIS EDEMA (pitting, non-pitting) ABDOMINAL -
A Diagnostic Conundrum
Open Access Case Report DOI: 10.7759/cureus.16832 Leucocytoclastic Vasculitis, Cryoglobulinemia, or Plasma Cell Leukemia: A Diagnostic Conundrum Hycienth Ahaneku 1 , Ruby Gupta 1 , Nwabundo Anusim 1 , Chukwuemeka A. Umeh 2 , Joseph Anderson 3 , Ishmael Jaiyesimi 1 1. Hematology and Oncology, Beaumont Health, Royal Oak, USA 2. Internal Medicine, Beaumont Health, Hemet, USA 3. Hematology and Oncology, Beaumont Health, Royal Oak, USA Corresponding author: Hycienth Ahaneku, [email protected] Abstract Plasma cell leukemia is rare and could be life-threatening. Even rarer and equally life-threatening is cryoglobulinemia. Both of them occurring together paints a grim clinical picture. We present the case of a 63-year-old male with plasma cell leukemia complicated by cryoglobulinemia with skin lesions. The report briefly reviews the clinical and diagnostic characteristics of plasma cell leukemia and well as available treatment options. It also highlights the need to consider non-chemotherapy-based regimens and clinical trials in the care of plasma cell leukemia patients. Categories: Oncology, Rheumatology, Hematology Keywords: plasma cell leukemia, cryoglobulinemia, multiple myeloma, bortezomib, lenalidomide, autologous stem cell transplant Introduction Plasma cell dyscrasias are a group of hematologic malignancies characterized by clonal proliferation of plasma cells. They cover a spectrum ranging from the premalignant monoclonal gammopathy of undetermined significance (MGUS) to the more aggressive multiple myeloma (MM) and plasma cell leukemia (PCL). PCL is often seen as the most aggressive plasma cell neoplasm. PCL accounts for less than 4% of plasma cell neoplasms and its population incidence is about one in a million, making it one of the rarest of plasma cell neoplasms [1].