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Anemia, hemolytic [keywords]
- Pulmonary Parenchymal Disease in a Patient With Chronic Lymphocytic Leukemia. [JOURNAL ARTICLE]
- Chest 2012 Oct 1; 142(4_MeetingAbstracts):563A.
SESSION TYPE: Cancer Case Report Posters IIPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION:Chronic Lymphocytic Leukemia (CLL) is a common haemotologic malignancy which can affect multiple organ systems. Pulmonary complications are most commonly infectious in nature, but here we present a case of parenchymal leukemic infiltratation.
CASE PRESENTATION:An 80-year-old man with history of chronic lymphocytic leukemia (CLL) diagnosed in 1983 presented to our institution with complaints of progressive cough, weight loss, and intermittent fever for several months. Prior to his current presentation, his CLL was indolent, not requiring specific treatment. Six months before his respiratory illness, he developed autoimmune hemolytic anemia requiring three months of steroids. Following completion of a steroid taper, he developed progressive respiratory complaints. His chest radiograph showed persistent right lung infiltrate, for which he had received multiple courses of antibiotics by his primary care physician for presumed pneumonia over the subsequent two months without response. At the time of presentation, the patient's white blood cell count was 5.8 X 103/mcL with 51.4% lymphocytes. A chest CT was obtained and showed right hilar and subcarinal lymphadenopathy as well as an ill-defined right hilar soft tissue mass with airway narrowing. He underwent a fiberoptic bronchoscopy which confirmed narrowing and erythema at the right middle lobe takeoff. Cytologic brushing, needle aspirations, and endobronchial biopsy were taken at this site which showed mucosal involvement with homogenous, mature, CD 20, CD5, and CD23 positive lymphocytes consistent with small lymphocytic lymphoma (SLL). Respiratory cultures were negative for infectious etiologies to include fungal organisms, acid-fast bacilli and Pneumocystis jiroveci. A PET scan revealed hypermetabolism in the right hilar mass and airspace disease. The patient was diagnosed with pulmonary SLL and referred to the oncology service. He was treated with radiation to the right hilar mass.
DISCUSSION:CLL is a common adult hematological malignancy accounting for approximately 30% of leukemias. The majority of pulmonary disease seen in CLL is caused by infectious complications. Pulmonary parenchymal involvement is much less common and typically occurs in patients with advanced disease. Rarely pulmonary involvement may be the sole or major manifestation of CLL. We present a case of a persistent pulmonary infiltrate in indolent CLL with biopsy proven pulmonary involvement as the major manifestation of disease.
CONCLUSIONS:This case emphasizes that clinicians should maintain suspicion for direct involvement of disease in leukemic patients presenting with persistent respiratory complaints and imaging abnormalities.1) Ahmed, S, et al. "Pulmonary Complications in Chronic Lymphocytic Leukemia," Cancer. 1Nov 2003; Vol 98(9). 1912-19172) Berkman N, Polliack A, et al. Pulmonary involvement as the major manifestation of chronic lymphocytic leukemia. Leuk Lymphoma. 1992; 8(6): 495-9.DISCLOSURE: The following authors have nothing to disclose: Sean Roark, Christopher King, John ShernerNo Product/Research Disclosure InformationWalter Reed National Military Medical Center, North Bethesda, MD.
- A Rare Case of Primary Body Cavity Effusion Lymphoma (PEL) Presenting as Severe Hemolytic Anemia. [JOURNAL ARTICLE]
- Chest 2012 Oct 1; 142(4_MeetingAbstracts):577A.
SESSION TYPE: Cancer Case Report Posters IIPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION:PEL is a rare form of Non-Hodgkin lymphoma that involves serous body cavities with lymphomatous effusions in absence of lymphadenopathy or organomegaly. Extracavity involvement is a rarity and only two cases have been described since its recognition in 1995. We report PEL presenting with hemolytic anemia, hepatomegaly and abdominal lymphadenoapthy and bowel wall thickening.
CASE PRESENTATION:A 53 yr old male with HIV on ART presented with sudden onset of generalized weakness. On presentation he was found to be in shock requiring intravenous fluids, vasopressors and blood transfusions. Examination showed pallor, jaundice and dry mucous membranes. He had decreased air entry bilaterally on auscultation as well as increased abdominal girth and hepatospleenomegaly. His initial labs revealed Hb of 3.4. Coombs test was consistent with hemolytic anemia. CT Chest showed bilateral pleural and pericardial effusion. CT abdomen showed hepatomegaly, adenopathy and ascites. EGD showed ulcerated nodule in gastric body and Colonoscopy showed thickening in transverse Colon. Gastric nodule biopsy and colon biopsy was consistent with gastric mucosa and colonic mucosa involvement by primary effusion lymphoma.
DISCUSSION:PEL is characterized by a lymphomatous body cavity effusion presenting as pleural/ pericardial effusion and acsites, in absence of a solid tumor mass. It is typically present in HIV patients and associated with Kaposi's sarcoma-associated herpes virus/human herpes virus 8 (KSHV/HHV8). It is characterized by liquid growth in fluid-filled body spaces. PEL tends to remain localized to the serous body cavities with no formation of solid tumor masses. It is composed of postgerminal center B cells, which typically display a non-B, non-T phenotype consistent with late stages of B-cell differentiation. HHV-8/KSHV is thought to play a major role in PEL pathogenesis via expression of several viral genes, which have the potential to affect B-cell growth. EBV has been seen in 70% of these cases.
CONCLUSIONS:Classical presentation of PEL is pericardial effusion, pleural effusion and ascites. Diagnosis is based on cytological analysis of the pleural effusion which reveals a high-grade lymphoma with round nuclei, prominent nucleoli and abundant cytoplasm. PEL does not necessarily limited to serous cavity and tends to involve GI tract. It can also cause hemolytic anemia, diarrhea, lymphadenopathy and hepatoslenomegaly. Presence of HHV-8/KSHV as well as EBV can help in differentiating this entity in high risk patients.1) Primary effusion lymphoma: a liquid phase lymphoma of fluid-filled body cavities. Gaidano G. Carbone A. Advances in Cancer Research. 80:115-46, 2001.DISCLOSURE: The following authors have nothing to disclose: Ameer Rasheed, Viswanath Vasudevan, Farhad ArjomandNo Product/Research Disclosure InformationThe Brooklyn Hospital Center, Brooklyn, NY.
- Severe Hypoxemic Respiratory Failure and Thrombotic Thrombocytopenic Purpura. [JOURNAL ARTICLE]
- Chest 2012 Oct 1; 142(4_MeetingAbstracts):317A.
SESSION TYPE: Critical Care Case Report PostersPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION:Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterized by hemolytic anemia and thrombocytopenia. Common microthrombi-related ischemic symptoms include cerebral, renal, cardiac, and gastrointestinal; reports indicate that dyspnea is an uncommon complaint and severe, life-threatening hypoxemia in the setting of TTP is extremely rare (1).
CASE PRESENTATION:A 24 year old black male developed altered mental status while exercising, culminating in syncope. Paramedics found him awake but confused. In the emergency department, patient was intubated due to altered mental status. The blood pressure was 241/137, oxygen saturation of 100%with clear lungs. Hemoglobin was 9.3g/dL, platelet count 46,000/ mm3, and peripheral smear showed 30 schistocytes/HPF. CT brain showed diffuse cerebral edema; MRI revealed bilateral cortical and leptomeningeal enhancement. Upon arrival to the tertiary center, patient remained hypertensive 168/110, heart rate 140 with temperature 38.1 C. Platelet count decreased to 22,000/ mm3, LDH 2105 u/L, and blood, urine and CSF studies were all negative for infection. Shortly after arrival gradual worsening hypoxemia ensued. Chest x-ray showed no infiltrate. PaO2 on blood gas was 42-45 mmHg despite paralytics and PEEP 15-20cmH2O on the ventilator; inhaled nitric oxide was ordered to bedside. Emergent plasma-exchange and steroids were instituted for presumptive TTP. Oxygenation improved with plasma-exchange and extubation occurred early morning day 3. Platelet counts had normalized to 210,000/mm3. Malignant hypertension was considered as the etiology of the thrombotic microangiopathy. However, significant fall of platelet counts from day 6 through day 8 of hospitalization with lack of hypertensive pressures necessitated repeat plasma-exchange and course of steroids resulting in rapid improvement and remission. ADAMST13 levels were <5% with presence of inhibitor.
DISCUSSION:A few case reports and series have proposed respiratory failure as a feature of TTP (2-3). However, confounding factors included co-diagnoses known to mimic findings of TTP and respiratory failure. A large patient registry indicates that on presentation, pulmonary symptoms are infrequent and significant infiltrates on chest x-ray are rare (1).
CONCLUSIONS:Our patient met clinical and laboratory criteria for the diagnosis of TTP having severe hypoxic respiratory failure without significant pulmonary infiltrates. We hypothesize that our patient's respiratory failure was related to occlusion of the pulmonary microvasculature with resolution as appropriate therapy was provided. Profound hypoxemia without pulmonary infiltrates has not been widely reported as part of the TTP symptom complex.1) George, James. Blood 2010;116:4060-40692) Panoskaltsis et al. Am Journal of Hematology 2000; 65:50-553) Chang et al. Am Journal Medical Sciences 2001; 321(2):124-128DISCLOSURE: The following authors have nothing to disclose: Timothy Nokes, Ahmed Awab, James George, Qiaofang ChenNo Product/Research Disclosure InformationUniversity of Oklahoma Health Science Center, Oklahoma City, OK.
- Tumor Lysis Syndrome in Small Cell Cancer of Prostate. [JOURNAL ARTICLE]
- Chest 2012 Oct 1; 142(4_MeetingAbstracts):626A.
SESSION TYPE: Cancer Cases IPRESENTED ON: Monday, October 22, 2012 at 01:45 PM - 03:00 PM
INTRODUCTION:Prostate is a rare site for Extra Pulmonary Small Cell Carcinoma (EPSCC). We report the first case of Tumor Lysis Syndrome (TLS) that developed in Prostatic EPSCC. This case was complicated by methemoglobinemia and severe hemolytic anemia after rasburicase (recombinant urate oxidase) use.
CASE PRESENTATION:A 56-year-old African American with a metastatic small cell cancer of the prostate developed TLS within 2 day of initiation of chemotherapy. 15 days prior to admission, he had a biopsy of a 1.7 cm hypodense nodule in the left lobe of the prostate that demonstrated poorly-differentiated small cell cancer (immunostain negative for PSA and TTF-1, positive for chromogranin, synaptophysin and CD56). His Serum PSA was 6.1. Extensive metastases to pelvic lymph nodes, liver (despite advanced alcoholic liver cirrhosis), lumber spine and lungs were noted, all with significant uptake on PET CT. Despite intravenous hydration and steroids, his serum creatinine, phosphate and uric acid increased from normal to 4.0, 7.4 and 16 respectively. On day 5, he received a dose of rasburicase. After rasburicase, his uric acid and phosphorus started to decline. However he became tachycardiac, tachypneic and hypoxemic (oxygen saturation in mid-80s on room air). He was noted to have methemoglobinemia (5.9%) for which he was given a dose of methylene blue and was transferred to intensive care unit. His hemoglobin dropped from 13 to 6.6 gm/dl with peripheral smear demonstrating numerous bite cells. Bilirubin increased to 21, LDH peaked at 3234 (baseline 160), haptoglobin <30, absolute reticulocyte count and reticulocyte index peaked at 206 and 82.4, respectively. He received 11 units via red cell exchange transfusion. Patient condition was stabilized and he was transferred to floor with Hemoglobin of 13 g/dl, total bilirubin of 4.4, methemoglobin of 0.6 and creatinine of 4.0.
DISCUSSION:TLS has not been reported in EPSCC of Prostate. This case highlights several important issues in prevention and treatment of TLS. Although our patient had normal baseline Creatinine, LDH and Uric Acid levels, he was at intermediate to high risk for developing TLS due to bulky tumor mass, extensive metastasis, urinary outflow-tract obstruction, high proliferative rate of malignant cells, chemo-sensitive malignancy and acidic urine. At risk patients should receive allopurinol or rasburicase prior to chemotherapy. Rasburicase treatment can induce hemolytic anemia in G6PD deficient cases but its routine measurement is not currently recommended. Methylene blue is not helpful in G6PD deficient cases and should not be used.
CONCLUSIONS:: Tumor Lysis Syndrome should be anticipated and treated with cautions in high risk patients.1) Howard SC, Jones DP, Pui CH. The tumor lysis syndrome. N Engl J Med 2011; 364:1844-18542) Bauters T, Mondelaers V, Robays H, et al. Methemoglobinemia and hemolytic anemia after rasburicase administration in a child with leukemia. Int J Clin Pharm 2011; 33:58-6DISCLOSURE: The following authors have nothing to disclose: Sikander Zulqarnain, Terrence Bradley, Spiro DemitisNo Product/Research Disclosure InformationSUNY Downstate Medical Center, Brooklyn, NY.
- Evans Syndrome and Granulomatosis With Polyangiitis: An Interesting Concomitant Occurrence. [JOURNAL ARTICLE]
- Chest 2012 Oct 1; 142(4_MeetingAbstracts):962A.
SESSION TYPE: Miscellaneous Cases IPRESENTED ON: Monday, October 22, 2012 at 01:45 PM - 03:00 PM
INTRODUCTION:Evans Syndrome and Wegener's granulomatosis are rare autoimmune conditions, both having annual incidence of less than 1 in 200,000(1). Evans syndrome is a combination of autoimmune hemolytic anemia and immune thrombocytopenia, while Wegener's granulomatosis is characterized by granulomatous vasculitis of the upper and lower respiratory tracts together with glomerulonephritis.
CASE PRESENTATION:A 42-year-old Caucasian female admitted to our hospital with acute febrile illness with thrombocytopenia. On further evaluation, she was diagnosed with Idiopathic thrombocytopenic purpura (ITP). Subsequently, patient was treated with steroids with favorable response. Six weeks later, she presented with nausea, vomiting and jaundice. Her hemoglobin was 5.2 g/dl with positive direct Coombs test. Elevated LDH, low haptoglobulin and splenomegaly were indicative of hemolysis. Evans Syndrome was diagnosed with the presence of autoimmune hemolytic anemia in the setting of ITP. Patient had partial response to steroids, IVIG, vincristine and splenectomy. Remission was achieved with the addition of cyclophosphamide following splenectomy. Thirteen years later, she presented with painless hematuria of four days duration. She also complained of persistent dry cough with symptoms of sinusitis for years. She was also being treated for urticarial vasculitis during the last three years. Further evaluation revealed proteinuria and acute onset renal failure. A chest radiograph revealed bilateral pleural effusion with bibasilar infiltrates. She was strongly positive for antimyeloperoxidase antibody and renal biopsy revealed focal necrotizing glomerulitis with epithelial crescent formation. At this point, a diagnosis of Wegener's granulomatosis was made(2). She was started on a cyclophosphamide along with prednisone, with induction of remission as indicated by an improvement in her renal function. Her symptoms of cough and sinusitis also improved with treatment.
DISCUSSION:Wegener's Granulomatosis is an uncommon disease characterized by granulomatous vasculitis of the respiratory tract with glomerulonephritis. Evans syndrome is an even more rare diagnosis, presenting with autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura. An association of autoimmune hemolytic anemia with Wegener's granulomatosis has been reported once(3).
CONCLUSIONS:To the best of our knowledge this is the first reported case of Wegener's granulomatosis concomitantly occurring with Evans syndrome.1) Norton A, et al. Management of Evans syndrome. Br J Haematol 2005; 132:125-1372) Guillevin L, et al. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum. 1999; 42:421-4303) Spiva DA, et al. Acute autoimmune hemolytic anemia due to a low molecular weight IgM cold hemolysin associated with episodic lymphoid granulomatous vasculitis. Am J of Med.1974; 56: 417-428DISCLOSURE: The following authors have nothing to disclose: Sandeep Sahay, Rajandeep Paik, Jaya Kala, Ziad KanaanNo Product/Research Disclosure InformationAkron General Medical Center, Akron, OH.
- Gain-of-function Lyn induces anemia: appropriate Lyn activity is essential for normal erythropoiesis and Epo-receptor signaling. [JOURNAL ARTICLE]
- Blood 2013 May 21.
Lyn is involved in Erythropoietin (Epo) -receptor signaling and is important for erythroid homeostasis. However, downstream pathways influenced following Lyn activation and their significance to erythropoiesis remain unclear. To address this, we determined the consequences a gain-of-function Lyn mutation (Lyn(up/up)) on erythropoiesis and Epo-receptor signaling. Adult Lyn(up/up) mice were anemic, with dysmorphic red cells (spherocyte-like, acanthocytes) in their circulation, indicative of hemolytic anemia and resembling the human disorder chorea acanthocytosis. Heterozygous Lyn(+/up) mice became increasingly anemic with age, indicating that the mutation was dominant. In an attempt to overcome this anemia, extramedullary erythropoiesis was activated. As the mice aged the relative levels of different immature erythroid populations changed, indicating compensatory mechanisms to produce more erythrocytes were dynamic. Major changes in Epo signaling were observed in Lyn(+/up) erythroid cell lines and primary CD71+ Lyn(up/up) erythroblasts, including significant alterations to the phosphorylation of Lyn, the Epo receptor, JAK2, STAT5, GAB2, Akt and FoxO3. As a consequence of altered Lyn signaling, Lyn(+/up) cells remained viable in the absence of Epo, but displayed delayed Epo-induced differentiation. These data demonstrate that Lyn gene dosage and activity is critical for normal erythropoiesis; constitutively active Lyn alters Epo signaling which in turn produces erythroid defects.
- Hematological and Genetic Predictors of Daytime Hemoglobin Saturation in Tanzanian Children with and without Sickle Cell Anemia. [Journal Article]
- ISRN Hematol 2013.:472909.
Low hemoglobin oxygen saturation (SpO2) is common in Sickle Cell Anemia (SCA) and associated with complications including stroke, although determinants remain unknown. We investigated potential hematological, genetic, and nutritional predictors of daytime SpO2 in Tanzanian children with SCA and compared them with non-SCA controls. Steady-state resting pulse oximetry, full blood count, transferrin saturation, and clinical chemistry were measured. Median daytime SpO2 was 97% (IQ range 94-99%) in SCA (N = 458), lower (P < 0.0001) than non-SCA (median 99%, IQ range 98-100%; N = 394). Within SCA, associations with SpO2 were observed for hematological variables, transferrin saturation, body-mass-index z-score, hemoglobin F (HbF%), genotypes, and hemolytic markers; mean cell hemoglobin (MCH) explained most variability (P < 0.001, Adj r (2) = 0.09). In non-SCA only age correlated with SpO2. α-thalassemia 3.7 deletion highly correlated with decreased MCH (Pearson correlation coefficient -0.60, P < 0.0001). In multivariable models, lower SpO2 correlated with higher MCH (β-coefficient -0.32, P < 0.001) or with decreased copies of α-thalassemia 3.7 deletion (β-coefficient 1.1, P < 0.001), and independently in both models with lower HbF% (β-coefficient 0.15, P < 0.001) and Glucose-6-Phosphate Dehydrogenase genotype (β-coefficient -1.12, P = 0.012). This study provides evidence to support the hypothesis that effects on red cell rheology are important in determining SpO2 in children with SCA. Potential mechanisms and implications are discussed.
- Autoimmune cytopenias in chronic lymphocytic leukemia. [Journal Article]
- Clin Dev Immunol 2013.:730131.
The clinical course of chronic lymphocytic leukemia (CLL) may be complicated at any time by autoimmune phenomena.The most common ones are hematologic disorders, such as autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). Pure red cell aplasia (PRCA) and autoimmune agranulocytosis (AG) are, indeed, more rarely seen. However, they are probably underestimated due to the possible misleading presence of cytopenias secondary to leukemic bone marrow involvement or to chemotherapy cytotoxicity. The source of autoantibodies is still uncertain, despite the most convincing data are in favor of the involvement of resting normal B-cells. In general, excluding the specific treatment of underlying CLL, the managementof these complications is not different from that of idiopathic autoimmune cytopenias or of those associated to other causes. Among different therapeutic approaches, monoclonal antibody rituximab, given alone or in combination, has shown to be very effective.
- Peritoneal EMH in a Dog with Immune-Mediated Hemolytic Anemia. [JOURNAL ARTICLE]
- J Am Anim Hosp Assoc 2013 May 20.
Extramedullary hematopoiesis (EMH) is the process by which normal blood cells are produced outside the bone marrow. In humans, EMH effusions are rare and are characterized by the presence of megakaryocytes, immature erythrocytes, immature leukocytes, or combinations of those cells. To the authors' knowledge, this is the first report to describe a case of peritoneal EMH effusion in a dog. A 5 yr old castrated male shorthaired dachshund presented with a 2 day history of pigmenturia and inappetence. A complete blood count revealed regenerative anemia with marked agglutination, spherocytosis, and an acute inflammatory leukogram characterized by a neutrophilia, regenerative left shift, and monocytosis. Ultrasound-guided aspiration of peritoneal effusion yielded a sample of high nucleated cellularity predominantly composed of mature and immature neutrophils and erythroid precursor cells. The patient was diagnosed with primary immune-mediated hemolytic anemia with concurrent EMH peritoneal effusion. The following case description and discussion explore the clinical findings associated with the unusual effusion and outline the possible pathogenesis by which the EMH effusion may have arisen in the dog.
- MRI Findings in a Dog with Kernicterus. [JOURNAL ARTICLE]
- J Am Anim Hosp Assoc 2013 May 20.
A severe increase in total bilirubin coincided with a decline in neurologic status to comatose in a 9 yr old spayed female mixed-breed dog being treated for immune-mediated hemolytic anemia. MRI of the brain was performed to investigate potential causes for the neurologic signs. MRI revealed bilaterally symmetrical hyperintensities within the caudate nuclei, globus pallidus, thalamus, deep cerebellar nuclei, and cortical gray matter on T2-weighted and fluid-attenuated inversion recovery (FLAIR) sequences, which coincided with areas of bilirubin deposition and neuronal necrosis (kernicterus) identified on necropsy examination. This is the second case report of an adult dog exhibiting kernicterus, and the first report to document MRI findings associated with that condition. Kernicterus is an uncommonly reported complication of hyperbilirubinemia in dogs, but is potentially underreported due to difficulties in recognizing subtle lesions and distinguishing kernicterus from other potential causes of neurologic abnormalities with readily available antemortem tests. MRI may be helpful in supporting the diagnosis of kernicterus.