Polycythemia Vera in a Dog Presenting With Uveitis

2003 ◽  
Vol 39 (4) ◽  
pp. 355-360 ◽  
Author(s):  
Heather E. Gray ◽  
Claire M. Weigand ◽  
Nancy B. Cottrill ◽  
A. Michelle Willis ◽  
Rhea V. Morgan

A 2-year-old, castrated male, mixed-breed dog presented with a 1-month history of red eyes and intermittent vomiting and a 2-week history of polyuria and polydipsia. Bilateral anterior uveitis and active chorioretinitis in the left eye were found on ophthalmic examination. Complete blood counts demonstrated evidence of an increased red blood cell mass. Thoracic and abdominal radiographs, abdominal ultrasonography, and Doppler echocardiography were unremarkable. Serum erythropoietin level was low-normal, consistent with a diagnosis of polycythemia vera. Resolution of all systemic and ocular signs occurred, and remission was achieved following phlebotomy and treatment with oral hydroxyurea.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4960-4960
Author(s):  
Thein H. Oo ◽  
Sumit Gaur ◽  
Janet Tierney ◽  
Alan Ashare ◽  
Robert Weinstein

Abstract Red blood cell mass (RCM) elevation is the sine qua non for diagnosis of polycythemia vera (pvera). The Polycythemia Vera Study Group (PVSG) employed therapeutic phlebotomy to lower the HCT to <55% as initial therapy. In the current era of frequent blood testing, RCM is usually requested based only on a high HCT often <55%. Thus the natural history of pvera may be changing if it is being diagnosed at an earlier stage. We reviewed 101 consecutive patients referred in 2002–2003 for RCM testing to characterize RCM requests from a large community referral base. There were 61 men and 40 women with mean HCT<55% (Table 1). 20 had lung disease or were smokers, 3 presented with arterial or venous thrombosis, 7 had cancer, 2 had renal cysts. HCT of men was higher than HCT of women if RCM was normal, but HCT were equivalent between genders if RCM was elevated. HCT was higher in men (p=0.0010) and women (p<0.0001) if RCM was elevated versus normal. HCT elevation was similar in secondary polycythemia vs pvera in men (51.0±4.02 vs 54.3±6.23; p=0.3397) or women (50.5±4.80 vs 49.3±2.12; p=0.4762). Table 1. HCT of patients referred for RCM measurement Gender Whole Group Normal RCM High RCM n HCT n HCT n HCT HCT shown as Mean (Median) ±SD Male 61 48.3 (47.8) ±4.89 45 46.9 (47.2) ±4.0 16 52.1 (51.3) ±5.16 Female 40 45.0 (43.8)± 4.72 32 43.3 (42.5) ±3.69 10 50.0 (50.0) ±3.84 p value 0.0001 <0.0001 0.4932 We examined whether, despite a lower presenting HCT, those with normal RCM demonstrated PVSG criteria that justified referral to our Nuclear Medicine department. Only 7 of the 75 patients with normal RCM met at least 2 of the PVSG “B” criteria or had splenomegaly, thus might have been diagnosed with pvera had RCM been high (Table 2). Table 2. 2 PVSG “B” criteria or splenomegaly and normal RCM Gender WBC ≥12K PLT ≥400K B12 ≥900 ≥ LAP 100 Splenomegaly M 14.4 413 640 158 “normal” M 12.1 585 1336 53 “normal” M 9.4 428 1139 68 “normal” M 8.6 132 1343 15 cm M 8.2 312 492 186 17 cm F 18.2 582 719 23 “normal” F 6.3 226 318 “enlarged” F 7.1 146 813 15.3 cm Overall, 10 of 40 women and 16 of 61 men had an elevated RCM: 4 women and 5 men with polycythemia vera; 6 women and 11 men with secondary polycythemia. One other man was diagnosed with polycythemia vera on the basis of a borderline elevated RCM (33.8 ml/kg), normal O2 Sat on room air, popliteal artery thrombosis, and 3 “B” criteria. Serum erythropoietin (EPO) was low at 2.6 mU/ml (ref range 4–16). He was receiving therapeutic phlebotomy for hereditary hemochromatosis when his RCM was measured. He was the only patient with pvera whose EPO was low (three others had normal EPO levels). The 11 patients with secondary polycythemia who had EPO measured had normal levels. In summary, the HCT was the primary criterion for RCM testing for 2/3 of these patients. Only 5 presented with HCT>55%; mean HCT was ~50% in patients with elevated RCM. The bottom quartile HCT of women with elevated RCM in our patient population was 48.7%. This is a sensitive “cut-off” for finding an elevated RCM (p<0.0001, Chi square with Yates correction). We conclude that patients are referred for RCM testing when a high HCT is found, but at HCT far below the original PVSG parameters. Therefore polycythemia vera is now diagnosed earlier and in mostly asymptomatic patients. A normal EPO level does not rule out a diagnosis of pvera. A HCT < 48.7% may not warrant the measurement of RBC mass.


2021 ◽  
Vol 51 (11) ◽  
Author(s):  
Ellen Bethânia de Oliveira Cavalcanti ◽  
Alana Carmela Ferrareis Cerqueira ◽  
Beatriz Barbosa Kaiser ◽  
Thieissa Moraes Venturotti ◽  
Cynthia Brandão da Costa ◽  
...  

ABSTRACT: Pancreatic cysts are rare in both humans and animals. They are defined as an enclosed structure externally surrounded by a capsule, internally coated with a cuboidal epithelium and filled with liquid or semi-solid content. This case described the clinical and pathological characteristics of a pancreatic cyst in a feline. A mixed breed cat with a history of recurrent vomiting was attended. Physical examination revealed pain on abdominal palpation. Abdominal ultrasonography showed a cystic, anechoic structure with well-defined edges located in the left cranial abdomen and in close contact with the duodenum and pancreas. Partial pancreatectomy was performed. Microscopically, the structure was surrounded by fibrous material, coated with cuboidal to columnar epithelium, and containing eosinophilic material. Although, pancreatic cyst is rare in animals, they should be included in the differential diagnosis of causes of vomiting in young cats.


Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4978-4978
Author(s):  
Joshua J. Goldman ◽  
Katherine Vandris ◽  
Fernando Adriano ◽  
Michael Bourla ◽  
Richard T. Silver

Abstract Abstract 4978 A low serum-erythropoietin (S-epo) level is a minor criterion of the World Health Organization (WHO) recommendations for diagnosing polycythemia vera (PV) even though previous studies indicate that a normal level does not always rule out PV. We wished to determine whether a normal S-epo level correlates with a low JAK2V617F mutant allele burden and a low phlebotomy (PHL) rate, since the relationship between S-epo level, JAK2V617F allele burden, and PHL rate heretofore has not been reported. At diagnosis, we grouped the S-epo levels of 26 PV patients (pts) as follows: low (<5 U/l), normal (5-10 U/l), and high (>10 U/l). Of the 26 pts, 4 (15.4%) had normal S-epo levels, and 22 had low S-epo levels. The diagnosis of PV in pts with normal S-epo levels was made by quantitative JAK2V617F analysis and elevated Cr51 red cell mass (RCM). All pts had a bone marrow biopsy consistent with PV. We determined the number of phlebotomies required to maintain normal hematocrit (hct) levels ('42% women, '45% men) as an assessment of disease severity during the period prior to myelosuppressive therapy. We then examined the number of phlebotomies per year (PHL/yr) in relation to S-epo level at diagnosis. Of the 26 pts, 14 were treated with PHL-only for a mean of 15 months (mos) prior to beginning other therapies including anagrelide, hydroxyurea, imatinib, and rIFNa-2b. The number of PHL/yr were grouped into 4 categories: 0 (none), 1 – 5 (low), 6 – 10 (moderate), and >10 (high). Of the 14 pts, 3 had a normal S-epo level at diagnosis, all of whom had a low PHL requirement, median 3/yr. The other 11 pts had low S-epo levels at diagnosis and a median PHL requirement of 6/yr (7 moderate and 4 low). Thus, while a low S-epo level at presentation was usually associated with moderate PHL requirement, a normal S-epo level at diagnosis was always associated with a subsequently low PHL requirement during the period of observation (mean 15 mos). Of the 26 pts, 14 had quantitative JAK2 analyses done at diagnosis. The other 12 were diagnosed prior to 2002 and quantitative JAK2 determinations were not available. Of the 14 pts, 2 had a normal S-epo level and a low JAK2V617F mutant allele burden (1-25%). These 2 pts also had a low PHL requirement. Thus, these pts who had normal S-epo levels, had JAK2V617F allele burdens in the lowest quartile, and had low PHL rates. Of the remaining 12 pts, 5 had JAK2V617F allele burdens in the 2nd (25-50%) quartile, 5 in the 3rd (50-75%) quartile, and 1 each in the 1st and 4th quartiles. We conclude (1) as previously reported, about 15% of pts with PV present with normal S-epo levels at diagnosis, suggesting a limitation of this WHO criterion, and (2) those pts with a low JAK2V617F allele burden and a normal S-epo level required fewer PHL/yr, all suggesting a more benign phenotype. This may provide a useful prognostic tool for patients with PV. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4078-4078
Author(s):  
Brady L Stein ◽  
Zheng Zhou ◽  
Jerry L. Spivak ◽  
Alison R. Moliterno

Abstract Introduction The 2005 identification of the JAK2 V617F mutation ushered in a new era of discovery for Polycythemia Vera (PV), allowing for its molecular classification, improved diagnostic capabilities, and a potential for targeted therapy. In this era, aspirin has been shown to lower the risk of thrombosis, the hematocrit (hct) target has been validated, and there has been renewed interest in interferon (IFN). These changes in diagnosis (dx) and therapy may impact the natural history of PV in its modern era. To this end, we analyzed 399 PV patients (pts) diagnosed in two eras (pre-2005 and post-2005) from two tertiary centers. (JHH, N=294; NU, N=105). Methods Pts were seen between 2005 and 2013. Testing to verify PV included an absolute erythrocytosis, JAK2 status, erythropoietin assay, red blood cell mass testing, and bone marrow biopsy. Pts were diagnosed by treating physicians using criteria relevant to the era of dx, between 1968 and 2013. Pts in each era were compared with regard to differences in demographic and clinical factors. The prevalence of myelofibrotic (MF) and leukemic (AML) transformation, as well as deaths was also determined. Results Of 105 NU and 294 JHH pts, 45 (43%) and 113 (38%) were diagnosed post-2005 (p=0.43). Pre-2005 and post-2005 pts were similar in age at dx at NU (52 vs. 57 yrs) and JHH (53 vs. 59 yrs). As expected, median disease duration was longer in pre-2005 NU (12 vs. 3 yrs) and JHH pts (9 vs. 2 yrs). 52% and 60% of pre-2005 pts, and 62% and 60% of post-2005 pts were women at NU (p=0.28) and JHH (p=1), respectively. JAK2 V617F testing was positive 33/37 (89%) and 41/44 (93%) of pre-2005 and post-2005 NU patients. At JHH, JAK2 V617F was positive in 286/294 (97%); JAK2 exon 12 was positive in 6/294 (0.02%). RBC mass testing was infrequently done at NU (9/105); only 2 were performed post-2005. At NU, pre-2005 and post-2005 pts had similar median leukocyte counts (8.9 and 8.7 x 109/L) and hct below 45% (40.5 vs. 42.9%). The prevalence of current anti-platelet and hydroxyurea use was similar at NU (pre-2005: 79%, 49% vs. post-2005: 82%, 48%, respectively). 5% and 11% of pre-2005 and post-2005 pts were on IFN. At NU and JHH, 29% and 27% of pre-2005 and 18% and 21% of post-2005 pts had thrombosis (pre vs. post: p=0.2 for NU; p=0.26 for JHH). Thrombosis in pre-2005 NU pts occurred at a median of 8 yrs from dx compared to post-2005 pts that frequently had events at presentation (median 0 yr; p=0.017). Similarly, fewer pre-2005 JHH pts had events at presentation compared to post-2005 pts (19/49 (39%) vs. 16/24 (67%); p=0.025). At NU, 19/105 (18%) developed MF, at a median of 12 yrs. Only 2/19 (11%) occurred in post-2005 pts, due to shorter disease duration. At JHH, 49/294 (15%) developed MF at a median of 11 yrs, but only in 4/49 (8%) post-2005 pts. At NU and JHH, 5/105 (5%) and 11/294 (4%), developed AML at a median of 15 and 8 yrs from dx. 1 and 4 post-2005 NU and JHH pts developed AML. At NU and JHH, 20/105 (19%) and 42/294 (14%) died. At NU, 16 were pre-2005 pts and 4 were post-2005 pts. At JHH, 35 were pre-2005 pts and 7 were post-2005 pts. AML-deaths were overrepresented in the post-2005 pts (25% NU, 57% JHH). Conclusion Remarkably, the epidemiology and natural history of PV was quite similar between two eras of diagnosis and between two large tertiary centers. Age at dx in pre and post-2005 patients was similar, arguing against a notion that pts are diagnosed earlier in adulthood. At NU, hct was typically below 45%, implying adherence to a recently validated target and aspirin use was prevalent regardless of the era of dx. Regardless, thrombosis at presentation was more common in modern era PV patients, whereas pre-2005 patients had more thrombotic events after an official PV diagnosis. As expected, time-dependent complications such as MF and death were more common in pre-2005 pts, and occurred within the 2nd decade of disease. MF was rare in post-2005 pts, who remain in their 1st decade of disease. Overall, AML was rare, but each cohort still had 1st decade transformations (post-2005 pts), over-representing deaths in this group. While the JAK2 mutation discovery has modernized PV dx, the natural history of PV has not changed. It is in this modern era of PV that we will learn whether or not new (JAK-inhibitors) and renewed (IFN) therapies can prevent or delay onset of feared time-dependent complications. Disclosures: No relevant conflicts of interest to declare.


2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A109-A110
Author(s):  
Marie Guruli ◽  
Vitaly Kantorovich

Abstract Adrenal infarct as a presenting finding in Polycythemia Vera (PV) Marie Guruli, Vitaly Kantorovich Background: While hemorrhagic adrenal infarcts, usually bilateral, have been described in hematologic malignancies, usually PV, no hemorrhagic variant are exceedingly rare. Here we describe a single case of unilateral nonhemorrhagic adrenal infarct. Case Presentation: 30-year- old male with past medical history of type 1 diabetes on insulin, presented with one day history of right upper quadrant (RUQ) abdominal pain. Initially pain was mild but slowly progressed to 10/10 intensity within the next several hours, felt like a sharp and stabbing pain with radiation up to the chest. Patient also reported to have difficulty breathing due to pain, along with nausea and vomiting. Laboratory results showed leukocytosis 11.2, H&H of 19.1/56.8, platelet count of 637, glucose of 283, alkaline phosphatase of 147. Abdominal ultrasonography reported nonspecific mild hepatomegaly and strongly suspected small thrombus within the IVC. Further work up included CTA study was negative for PE (pulmonary embolus), but MRI showed a filling defect in the suprarenal IVC along with right adrenal infarction. Patient was thermodynamically stable. Fortunately, cortisol was 21.3 and DHEA levels were 419, indicating no apparent effect on adrenal function. Patient underwent a bone marrow biopsy and was eventually diagnosed with PV. He was discharged in the stable condition with a close follow up with his endocrinologist for monitoring and repeat MRI for right adrenal infarct within the next few months. Conclusion: This case illustrates the importance to screen patients with PV who presented with similar clinical presentation for possible adrenal insufficiency. It also highlights the importance of undertaking imaging for careful consideration, close follow up to prevent life threatening complications and even death.


2003 ◽  
Vol 39 (1) ◽  
pp. 47-51 ◽  
Author(s):  
Christopher L. Horstman ◽  
Paul A. Eubig ◽  
Karen K. Cornell ◽  
Safdar A. Khan ◽  
Barbara A. Selcer

A 2-year-old, male, mixed-breed dog presented with a 12-day history of vomiting, depression, and weight loss after ingestion of industrial-strength wood glue containing diphenylmethane diisocyanate as its active ingredient. A diagnosis of gastric foreign body was made from survey abdominal radiographs. A large aggregate of solidified wood glue was surgically removed, and the dog recovered uneventfully. Fourteen other cases have been reported to the Animal Poison Control Center at the American Society for the Prevention of Cruelty to Animals (ASPCA). Eight of those 14 cases required surgical intervention. All cases recovered completely.


2021 ◽  
Vol 7 (2) ◽  
pp. 205511692110484
Author(s):  
Joana Tabanez ◽  
Samuel Beck ◽  
Colin Driver ◽  
Clare Rusbridge

Case summary A 10-year-old male neutered Russian Blue cat was presented with a 2-month history of progressive non-ambulatory paraparesis. Spinal MRI revealed a well-demarcated, compressive intradural extramedullary mass at the level of T1 vertebra. The mass had subtle hyperintensity on T2-weighted images, was isointense on T1-weighted images and had diffuse, marked enhancement following gadolinium administration. Neuroaxis MRI, including limited brain sequences, excluded other visible lesions. Thoracic and abdominal radiographs were unremarkable. The mass was resected via a dorsal C7–T2 laminectomy and durotomy. Histopathology revealed a neoplasm composed of columnar-to-polygonal cells forming bilayered palisading patterns with a few apical cilia. Three mitoses were noted in 10 high-power fields. This was consistent with an epithelial neoplasm and initially a metastatic adenocarcinoma was considered most likely. Full-body CT with contrast and including the brain found rhinitis but did not identify any additional neoplastic foci. Biopsies of the nasal cavity and fine-needle aspiration of the spleen and liver were unremarkable. On immunohistochemical evaluation, pan-cytokeratin and E-cadherin immunolabelling was observed; however, synaptophysin, thyroglobulin, chromogranin A and glial fibrillary acidic protein was not detected. This, along with the histological morphology and absence of a primary tumour, was compatible with an ectopic choroid plexus neoplasm. Follow-up performed at 3, 14 and 24 months postoperatively revealed neurological improvement without recurrence. Relevance and novel information We describe the presentation, histopathological and immunohistochemical features and outcome of a case of a rare ectopic choroid plexus neoplasm in the spinal cord of a cat.


2020 ◽  
Vol 56 (3) ◽  
pp. 185
Author(s):  
Alexandra Guillén ◽  
Matteo Rossanese ◽  
Emanuele Ricci ◽  
Alexander James German ◽  
Laura Blackwood

ABSTRACT Intravascular lymphoma (IVL) is a rare, high-grade, extranodal lymphoma characterized by selective proliferation of neoplastic lymphocytes within the lumen of small vessels. A 10 yr old female intact mixed-breed dog was presented with a 7 mo history of vomiting and anorexia. Physical examination revealed abdominal discomfort. Ultrasonography and endoscopy identified a submucosal gastric mass. Excision was performed by partial gastrectomy and histopathology and immunohistochemistry confirmed a T-cell IVL. The owner declined chemotherapy, and the dog was instead treated palliatively with prednisolone. Two months after surgery, vomiting recurred and abdominal ultrasonography revealed a large gastric ulcer with focal peritonitis. The dog was euthanized 4 mo after initial presentation and postmortem examination confirmed IVL recurrence in the stomach and an isolated nodule of neoplastic cells in the omentum. No involvement of other organs was found following histopathological examination. This is the first description of primary gastric intravascular lymphoma causing chronic vomiting in a dog.


2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Tatsuki Ishikawa ◽  
Katsunori Nakano ◽  
Masafumi Osaka ◽  
Kenichi Aratani ◽  
Kadotani Yayoi ◽  
...  

Abstract Background  Primary neuroendocrine tumors of the gallbladder (GB-NETs) are rare, accounting for 0.5% of all NETs and 2.1% of all gallbladder cancers. Among GB-NETs, mixed neuroendocrine–non-neuroendocrine neoplasms of the gallbladder (GB-MiNENs) are extremely rare. Case presentation We present the case of a 66-year-old woman who was referred to us for the management of a gallbladder tumor (incidentally found during abdominal ultrasonography indicated for gallbladder stones). The patient had no history of abdominal pain or fever, and the findings on a physical examination were unremarkable. Blood tests showed normal levels of tumor markers. Imaging studies revealed a mass of approximately 10 mm in diameter (with no invasion of the gallbladder bed) located at the fundus of the gallbladder. A gallbladder cancer was suspected. Therefore, an open whole-layer cholecystectomy with regional lymph nodes dissection was performed. The postoperative course was uneventful, and she was discharged on postoperative day 6. Pathological findings showed GB-MiNENs with invasion of the subserosal layer and no lymph node invasion (classified T2aN0M0 pStage IIA according to the Union for International Cancer Control, 8th edition staging system). Analysis of the neuroendocrine markers revealed positive chromogranin A and synaptophysin, and a Ki-67 index above 95%. Fourteen months after the operation, a local recurrence was detected, and she was referred to another hospital for chemotherapy. Conclusions  GB-MiNENs are extremely aggressive tumors despite their tumor size. Optimal therapy should be chosen for each patient.


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