BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is considered a rare disease. In the Philippines, there are currently no local registry for such rare disease thus clinical features that may be unique to the country is difficult to characterize.
OBJECTIVE: To characterize the TTP patients seen in our institution based on the demographic, clinical and laboratory profile of these patients.
RESULTS: A total of eight patients were described in this report. Median age was 38 years (range, 20-63) with a female predominance. All patients had neurologic  symptoms and microangiopathic hemolytic anemia. Minor neurologic symptoms (confusion, headache and dizziness) were more frequently observed. Renal dysfunction (serum creatinine >1.4mg/dL), fever (>38.0°C), and thrombocytopenia were observed in four patients (50%), seven patients (87.5%) and six patients (75%) respectively. Patients underwent median of three sessions of Plasma Exchange (range, two to 38). Of the six patients who received therapeutic plasma exchange, five were discharged alive.
CONCLUSION: Fifty percent of the patients in this report presented with the classic pentad. This feature should be kept in mind as TTP is a true hematopologic emergency. A high index of suspicion, prompt evaluation and treatment of patients are necessary because multi-organ thrombosis may not be reversible. If treatment is not urgently administered, Therapeutic Plasma Exchange is the management of choice and should be done immediately once TTP is recognized.

Human ; Male ; Female ; Adult ; Purpura, Thrombotic Thrombocytopenic ; Patients ; Thrombocytopenia ; Plasma Exchange

OBJECTIVES: To describe the clinical course and treatment response of a case of thrombotic thrombocytopenic purpura (TTP) after a month of therapeutic plasma exchange (TPE) and immunosuppressive agents and to review related published literature regarding TTP and its response to TPE and immunosuppressive agents.
CASE AND DISCUSSION: A 64-year-old female presented with fever, bicytopenia, change in sensorium, and seizure of one day duration. Metabolic panel showed normal electrolytes with normal brain imaging. Hematologic work-up showed anemia, thrombocytopenia and leukocytosis with normal differential count. Reticulocyte was elevated. Peripheral smear showed significant schistocytes with nucleated red blood cells and marked thrombocytopenia. Thrombotic thrombocytopenic purpura was the initial consideration with the fulfilment of four out of the pentad of TTP, namely: microangiopathichaemolytic anemia, thrombocytopenia, fever and neurologic symptoms. Therapeutic plasma exchange was immediately initiated with daily platelet count and Lactate dehydrogenase (LDH) determination to assess response. However, despite daily plasma exchanges and continuous plasma infusion, there was inadequate response. In this light, immunosuppressive agents were started in the following order: high dose methylprednisolone, weekly rituximab and intravenous cyclophosphamide. On the 31st hospital day, after daily TPE and combined immunosuppression, she achieved complete response with a platelet count of 170 X 109/L to 250 X 109/L (baseline-14 X 109/L) and LDH 227U/L (baseline-1,191 U/L).
CONCLUSION: This study presented a challenging case of TTP which was successfully treated with the standard of care together with the available adjunctive treatment options.

Human ; Female ; Middle Aged ; Plasma Exchange ; Purpura, Thrombotic Thrombocytopenic ; Rituximab ; Thymidine 5'-triphosphate ; Lactate Dehydrogenase 1 ; Isoenzymes ; L-lactate Dehydrogenase ; Anemia

INTRODUCTION: Ferbile neutropenia is considered a medical emergency and remains a major cause of morbidity and mortality among cancer patients. In this population, infections are often characterized by the lack of significant clinical findings during physical examination and a clear focus of infection, perhaps because of the inability to mount an adequate inflammatory response. It has long been recognized that any delay in treatment may result in a higher risk of mortality especially when caused by highly virulent pathogens such as Pseudomonas aeruginosa.
OBJECTIVE: To determine the clinical profile and disease outcome of the various infections an adult patient with leukemia who developed febrile neutropenia admitted at the University of Santo Tomas Hospital from January 1,2010 to July 31,2013.
METHODOLOGY: This is a three and a half year retrospective descriptive study. Medical records of all patients >18 old with leukemia, who were admitted and assessed to have infection which developed at the time of the neutropenia were included.
RESULTS: A total of 44 cases of leukemia with febrile neutropenia were reviewed. There was equal distribution between genders. Mean age was 39.64 years. Majority of patients had acute myelogenous leukemia (AML) [72.7%, (32/44)]. All patients had sepsis, where two, (4.5%) manifested with shock. Co-morbidities included hyperthyroidism (9.15%), diabetes milletus (DM) (6.8%), and hypertension (6.8%). Cefepime [40.9% (18/44)] and meropenem [31.8%, (14/44)] were the most coon antimicrobial agents used to treat neutropenic patients.
All of the patients had blood cultures, however, only 12, (27.3%) yielded positive results. Among those with negative culture results, 18.8% received antibiotics prior to collection of blood. Other specimens tested sputum and urine. Among those with positive results, the most common organisms isolated were E. coli [33.3%, (4/12)] followed by Enterobacter cloacae [16.7%, (2/12)]. Other organisms isolated include Aeromonas hydrophilia , Pseudomonas aeruginosa , Stenotrophomonas maltophilia , coagulase-negative straphylococci (CoNS), and viridans streptococci.
Nineteen (43.2%) patients had pneumonia, while five (11.4%) patients had urinary tract infection. Seventeen (38.6%) patients had no known focus of infection despite aggressive search. Culture positivity was observed more frequently among those with profound neutropenia [91.7% (11/12)] and those with Multinational Association for Supportive Care in Cancer (MASCC) score of less than 21 [100%, (6/6)].
Thirty seven (84%) of patients were discharged improved, (six 14%) died due to infection and one patients was discharged against medical advice. Mortality was high among those with profound neutropenia, (6/6, 100%) and those with MASCC score of less than 21 [100%, (6/6)].
CONCLUSION: Acute myelogenous leukemia cases comprised majority of febrile neutropenic patients. All patients had sepsis commonly involving the lungs. Co-morbidities included hyperthyroidism, DM and hypertension and few patients had prior use of antibiotics. Blood culture was positive in more than one fourth of the population. The most common organisms isolated were E. coli followed by Enterobacter cloacae. Mortality was high among patients with profound neutropenia and MASCC score of less than 21.

 

 

Human ; Male ; Female ; Aged ; Middle Aged ; Adult ; Aeromonas ; Anti-bacterial Agents ; Cephalosporins ; Coagulase ; Diabetes Mellitus ; Enterobacter Cloacae ; Escherichia Coli ; Fever ; Hypertension ; Hyperthyroidism ; Leukemia, Myeloid, Acute ; Stenotrophomonas Maltophilia ;