Objective:Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease. The primary aim was overall response rate (ORR) assessment in the treated patientsMethods:This retrospective study included 24 patients treated during 2006-2015. TTP patients with microangiopathic hemolysis (MAHA) and thrombocytopenia were included. We analyzed clinical features, laboratory characteristics and treatment outcomes of 24 TTP patients treated at our tertiary care center (KFMC).Results:Twenty-four TTP patients (18 females; 6 males) had a mean age of 33.5±13.9 years; 22(91%) had neurologic features, 7(29%) fever, 10(42%) renal impairment; 4(20.83%) cardiac manifestations; 22(91.7%) had triad with additional neurologic abnormalities; only 2(8.2%) had pentad of TTP. Majority (54.16%) had idiopathic TTP. All patients received therapeutic plasma exchange (TPE); 23(95.8%) received adjunctive corticosteroids and 13(54.2%) received rituximab either due to refractoriness to TPE on ~day7, or earlier. Twenty-one out of 24 (87.5%) achieved complete remission (CR) without any subsequent relapse. At 22 months (median, range 1-113), 20 patients (83.3%) are alive at the time of report. Three patients died during acute episode because of sever disease or delayed treatment and one died in CR.Conclusion:TPE, steroids and or rituximab was very effective in preventing high risk of mortality and achieving durable CR in 87.5% of patients. More awareness is needed for early diagnosis and early referral to centers with appropriate tertiary care facilities..
Introduction Respiratory viruses are an important cause of outbreaks of pneumonia in hematological malignancy patients. Recently, novel Middle East respiratory syndrome coronavirus (MERS-CoV) caused a cluster of life-threatening infections in Saudi Arabia (688 confirmed MERS-CoV infection cases with 282 deaths were reported to WHO by June 6, 2014 including 28% cases in HCW). Most patients had upper &/or lower respiratory tract symptoms but other features included abdominal pain, diarrhea, acute kidney injury & shock. Few hematology units were closed due to havoc. Here we report clinical features & outcome of 4 patients diagnosed at our unit during the peak period (mid-March through May 2015) including 2 who got chemotherapy (chemo) soon after recovery. Patient #1 A 62-yr-old male had free λ light chain multiple myeloma (MM) with spinal cord compression. After surgery & local radiation, he was sent to us with paraplegia & grade 4 infected sacral bedsore that needed inpatient care. He was started on CyBorD Cycle 2 on April 24, 2014. On May 2, he had a fever spike with shortness of breath (SOB) & cough. He was started on antibiotics. CXR revealed bilateral infiltrates & right sided pleural effusion. His O2 sat dropped & he needed CPAP. Oseltamivir was started & sputum was positive for MERS-CoV RT-PCR. He became afebrile with decreasing O2 requirement, CXR normalized & RT-PCR for MERS-CoV turned negative. Later he was able to receive 3rdcycle of CyBorD. Patient #2 A 65-yr-old lady came to us with B symptoms & huge organomegaly due to stage-IV DLBCL. On May 2, 2014 RCVP chemo was started. On day 6, she spiked fever with SOB, cough & was started on imipenem. CXR showed consolidation in right lower lobe. She needed 4L of O2/min. On May 10, 2014, she worsened with RR 32/min, O2 sat 79% on 15L O2/min & BP 79/47 mmHg. CXR revealed bilateral consolidation. She needed intubation & inotropic support in ICU. Vancomycin & oseltamivir were started & RT-PCR was positive for MERS-CoV on two nasopharyngeal swabs (NPS). LFT & RFT were normal but she continued to decline & died on May 13, 2014. Patient # 3 A 22-yr-old lady with past H/O AML t(8;21) was admitted on April 27, 2014 with 3 day H/O cough, fever & SOB. CXR had infiltrates in left lower lobe. She had severe pancytopenia & BMB confirmed relapsed AML. She had slightly raised LFT. Urine grew Ent. fecium. Antibiotics & voriconazole were used. She remained febrile over next 2 days. CT chest revealed extensive bilateral consolidation. She needed O2 up to 5L/Min for few days. RT-PCR for MERS-CoV was positive from NPS. She was initiated on oseltamivir. She became afebrile after 2 days & repeated RT-PCR for MERS-CoV was negative. Fludara, Ara-C (FA) chemo was started. She remained neutropenic for next 4 weeks but there was no recurrence of respiratory symptoms. BMB on day 28 of FA confirmed CR. CT chest revealed complete resolution of air space opacities. She was discharged with plan to undergo matched sibling donor Allo-HSCT. Patient #4 A 76-yr-old male with H/O HTN & CKD was diagnosed to have IgA κ MM. He was started on MPV chemo as inpatient due to logistic reasons. After 3 cycles of MPV, serum free κ chains decreased by 91% but remained on dialysis. On 21 April, 2014 he developed cough, SOB & fever. CXR revealed bilateral infiltrates & antibiotics were started. He worsened over next few days & CXR showed worsening bilateral consolidation. Eventually he needed intubation. He was treated with antimicrobials including voriconazole & oseltamir. RT-PCR for MERS-CoV was positive from NPS. Unfortunately he died few days later. Discussion: Patients with hematological malignancies are at increased risk of community & hospital-acquired infections. Recent outbreak of MERS-CoV infection has created a havoc among hematologists community. There is uncertainty about impact of MERS-CoV infection on continuation of chemo. We report 4 cases of hematological malignancies with MERS-CoV infection. Three of the 4 patients developed severe pneumonia & required intubation (2 died later) & one had milder form of pneumonia treated in isolation room. In addition to supportive care, all 4 received antimicrobials & oseltamivir. Chemo was safe soon after recovery from infection in the surviving 2 patients. We propose that during MERS-CoV epidemics, pneumonia can be treated with supportive care, antibiotics & oseltamivir. Chemo can be continued for the malignant disease soon after recovery. Further reports are needed to confirm our findings. Disclosures No relevant conflicts of interest to declare.
Introduction: Magnesium is the fourth most abundant cation in the body and the second most abundant intracellular cationafter potassium. Magnesium plays a fundamental role in many functions of the cell, including energy transfer, storage, and uses protein,carbohydrate, and fat metabolism; maintenance of normal cell membrane function; and the regulation of parathyroid hormone (PTH)secretion. Objective: To determine the frequency of hypomagnesaemia in patients presenting with acute coronary syndrome (ACS).Design: Cross -sectional study. Place and duration: Coronary Care Units and medical ward in Allied Hospital Faisalabad, from 26-01-2010 to 25-07-2010. Settings: The study was conducted in medical unit II and coronary care unit of Allied Hospital Faisalabad. SampleSize: Sample size was calculated by using WHO sample size calculator taking confidence level 95%, population proportion 7.7% andrequired precision 4%. Sample size n = 171. Sampling Technique: Non-probability consecutive sampling. Method: A total of 171patients fulfilling the criteria of ACS admitted in M-II and CCU were enrolled in the study, demographic details, history and clinicalexamination of the patients were recorded. Blood sample was collected in estimation of serum magnesium level. Results: In this study themean serum magnesium was 1.59 ±8.380 in males and 1.56±7.678 in females. Among the 171 acute coronary syndrome patients, 14(8.2%) were diagnosed with hypomagnesaemia. There were 8 (8.8%) male and 6(7.5%) female patients. 157 (91.8%) patients did nothave hypomagnesaemia out of 83 (91.2%) were male and 74 (92.5%) were female patients. There was male predominance. Male to maleratio was 1.33:1. Conclusions: The results showed that frequency of hypomagnesaemia in acute coronary syndrome was significantlyhigh and comparable to other studies. There was male preponderance. However, there was variation in the occurrence ofhypomagnesaemia in acute coronary syndrome. Early assessment of serum magnesium concentration is needed in acute coronarysyndrome in order to implement proper magnesium supplementation.
Acquired Hemophilia (AH) may lead to fatal bleeds. Relapse remains a major challenge as 1/3 of patients with AH do not achieve durable CR with immunosuppressive therapies (IST). Patients’ acceptance for cyclophosphamide (being a chemotherapeutic agent) for treating AH remains poor as compared to biological agent rituximab (R) and steroids (St). However, when fixed number of doses of R are utilized along with prolonged courses of St, considerable morbidity is seen. In quest of durable remission and prevention of relapse, we hypothesized that a strategy of targeting antibody producing B cells along with inhibitor (Inh) monitoring and titrated number of R doses earlier in the course of therapy with one additional dose beyond complete disappearance of Inh may result in better chances of prolonged remission and even cure. Here we report excellent outcome with our strategy used for three consecutive patients including a patient with multiple relapses and high titer Inh (819 BU) in whom all other therapies had failed to produce durable remission. Median time to CR from addition of R was 6 weeks (range 3-6). With a median follow up of 16-72 months none has relapsed yet. CASE #1 A student was diagnosed to have bleeding diathesis in November 2007 in Kuwait at the age of 13 years and was treated with FFP, FVIII, rFVIIa and St. He presented in December 2007 to pediatric hematology complaining of swellings in the muscles, hematuria, hematemesis, severe abdominal pain and bruising for the last 2 weeks. He had tenderness in hypogastrium and right iliac fossa, multiple skin bruises with multiple swellings over extremities. Eventually he was diagnosed to have AH with FVIII Inh of > 850 BU. He had intra-abdominal and multiple joint bleeds on imaging. He was managed with rFVIIa, IVIG, St and cyclophosphamide. Because of persistent low FVIII level (0.17%) he was given 4 doses of R along with prolonged steroid course. This resulted in normalization of FVIII level and Inh disappearance. However, soon he suffered 3 quick relapses with bleeding treated with 2 doses of R, St and IVIG. He returned in relapse with right thigh swelling, hematuria, and epistaxis in February 2013 (FVIII < 1 % and Inh 57.6 BU). He was treated again with rFVIIa and a dose of R. He was then referred to our team with Inh 115.2 BU. After giving him rFVIIa and starting St we added R on a weekly basis and prednisolone 1mg/kg daily. Inh disappeared after the 6th dose. He received the additional R dose (#7) on April 14, 2013. At this point, we could start immediate tapering of his 40 mg prednisolone dose and discontinued by July 2013. His FVIII level later rose to 200 % with undetectable Inh. He is off St since September 2013. He remains in CR after more than 16 months of his last dose of R. CASE #2 This 20-year-old lady presented to ER in March, 2013 with sudden onset swelling of the right hand, bluish discoloration and moderate wrist pain for 3 days. She gave past history of migratory joints swelling. Her mother had thrombocytopenia for the last 7 years. She had a tender warm swelling of right hand with bluish discoloartion, remarkably decreased ROM of the right wrist. She was admitted under internal medicine with possible diagnosis of CTD. Investigations revealed an ANC of 1.16x10^9/L; and an isolated prolonged PTT of 102.9 seconds that after mixing with pooled normal plasma (1:1) was 91.9 seconds. FVIII level was 0.20% and FVIII Inh was 22.4 BU. Based on further investigations a diagnosis of SLE with APLA and AH was made. rFVIIa was given, and by rheumatology team prednisolone 40 mg daily along with HCQ 400mg started on 17 March 2013 (prednisolone was tapered to discontinuation by October 2013). As bleeding manifestations continued, R 500mg weekly doses were started on 27th March 2013. After a few days of the 3rd dose, her Inh had disappeared and FVIII level had normalized. We gave her 4th dose after it according to our strategy and then started quick prednisone tapering. Patient remains in CR under follow up. CASE #3 A 28-year-old female was diagnosed to have acquired hemophilia after severe postpartum bleeding in February 2008 with FVIII Inh titer of 49.92 BU. After rFVIIa, with ongoing prednisolone her Inh level rose to 230 BU and she refused to consent for cyclophosphamide. She required 7 weekly doses of R after which Inh disappeared. Then one additional (8th) dose of R was given. Later she had an early abortion and then twin pregnancy in August 2012 without relapse. She remains in CR 6 years after her last treatment. Disclosures Off Label Use: Rituximab is a genetically engineered monoclonal antibody that targets a specific protein, known as CD20 found on the surface of normal and malignant B-lymphocytes. Use of Rituximab treatment for other than FDA approved indications include a rare condition of acquired factor VIII inhibitors in individuals without inherited hemophilia as an autoimmune phenomenon that may lead to life-threatening bleeding. Due to the low incidence rate of acquired inhibitors, published data consists of only case reports, and reviews.
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