INTRODUCTION

Pure red cell aplasia (PRCA) is defined as anemia in the presence of severe reticulocytopenia and absent or markedly decreased erythroid precursors in the marrow. When associated with another disease entity, it is classified as secondary acquired PRCA. A rare entity, thymolipoma, which constitutes 2% to 9% of thymic tumors has been associated with PRCA in some studies. The prevalence of thymolipoma among patients with myasthenia gravis reaches 43.8%. This paper presents the rare presentation of myasthenia gravis associated with thymolipoma and PRCA.

We present the case of a 64-year-old female who was diagnosed with myasthenia gravis and has been on maintenance pyridostigmine (Mestinon) for 12 years. She presented with symptoms of anemia and became transfusion requiring. Routine chest CT showed a thymic mass which was confirmed to be a thymolipoma during biopsy. Bone marrow studies confirmed the absence of erythroids, hence the diagnosis of secondary acquired PRCA.

There have been case reports associating PRCA with thymolipomas. Thymolipomas, which constitute 2% to 9% of thymic tumors are found among patients with myasthenia gravis, but the majority remains asymptomatic despite increasing tumor size. PRCA with associated thymic mass shows improvement of symptoms with tumor removal and immunosuppression. Oral corticosteroids result in a response rate of 39% while cyclosporine results in 77% response. This can be tapered off once response has been achieved.

This case report emphasizes the importance of early bone marrow studies among patients with myasthenia gravis presenting with sudden onset anemia.

Background: The field of medicine is constantly changing. Notable changes occur in the patterns of clinical practice, business of medicine, shift in demographics/generation of the health care workforce, emergence of sub-specialization; and advances in research and technology. These changes can affect the way young physicians establish their practice and this is an area not addressed by the medical education and training. Purpose: There is little data in the literature regarding the experience of young physicians in establishing clinical practice. This study was undertaken to answer the central question: Among physicians in the field of Internal Medicine with or without subspecialty who graduated from training in 2013 to 2018, what were the issues and challenges that they faced as they established their clinical practice in the urban or mixed setting? Methodology: Qualitative research-case study; Key informant interview was conducted among junior Internal Medicine consultants who satisfied the inclusion criteria. Data analysis used thematic analysis consisting of reading, writing notes, describing, and classifying transcripts according to categories and themes. Results: After a comprehensive analysis of narratives, five emergent themes surfaced: “Tough Days” (Period of figuring out the system; Need for Self-introduction; Few patients and Feelings of frustration and depression); “Torn and Divided” (Unpredictable work schedule and workload; Lack of time for issues outside career); “Temporary Debt” (Large start-up cost; Expensive maintenance); “Difficult but Tolerable” (Family support; Call-a-colleague; Debt of gratitude to mentors) and lastly “Dreams and To-do’s”. Conclusions Our respondents have experienced substantial challenges in starting clinical practice. Learning the ways of the healthcare business, effectively promoting oneself to the community, dealing with the emotional turmoil of having few patients, coming up with a strategic schedule and area of practice, and looking for funds and paying it back, were the challenges and experiences of these young medical specialists as they establish their careers in the urban and/or rural setting.
Private Practice

Introduction : Multiple myeloma (MM) causes generalized bone loss leading to lytic bone lesions and pathologic fractures. The increased osteoclast activity and reduced osteoblast function favors bone resorption and decreased bone formation. Vitamin D is vital in regulating calcium homeostasis and osteoclast-mediated bone resorption. Defi ciency of Vitamin D among MM patients may complicate bone mineralization problems and fractures. Objective: General Objective:To determine the status of Vitamin D in patients with multiple myeloma Specific Objective: To determine the levels of Vitamin D, intact parathyroid hormone and ionized calcium among MM patients. Methodology : This is a prospective, cross-sectional study which included patients who were 18 years old and above, male or female, diagnosed with MM at the University of Santo Tomas Hospital, with or without treatment. Excluded in the study were those with Vitamin D and calcium supplementation. Eligible subjects were extracted blood for Vitamin D assay, intact parathyroid hormone and ionized calcium. Results: A total of 22 patients with MM were included in the study. Sixteen patients (72.7%) had hypovitaminosis D. Among these sixteen patients, seven (31.8%) had Vitamin D defi ciency (Vitamin D levels <20 ng/mL [50 nmol/L]) and nine (40.9%) had Vitamin D insuffi ciency (levels of 21-29 ng/ mL [52.5-72.5 nmol/L]). Only 6 (27.3%) of them were found to have normal serum Vitamin D (levels of >29 ng/mL [>72.5 nmol/L]). The mean age (p=0.069), intact PTH (p=0.062) and ionized calcium (p=0.188) of the three groups of patients did not differ Conclusion : This study found a high incidence of Vitamin D defi ciency among MM patients seen at the University of Santo Tomas Hospital. Vitamin D defi ciency was independent of age, intact PTH and ionized calcium. It was more common in male subjects. Patients with hypovitaminosis D are at risk of having secondary hyperparathyroidism. Recommendation Vitamin D status should be determined among patients with MM. Early recognition and treatment of hypovitaminosis D will prevent the risk of having secondary hyperparathyroidism that can complicate skeletal-related events.
Multiple Myeloma ; Calcifediol ; Vitamins

Introduction : Monoclonal antibodies have revolutionized the treatment of immune-mediated infl ammatory diseases (eg, rheumatoid arthritis [RA], Crohn’s disease [CD], and psoriasis) as well as malignant diseases. Currently, there are about 100 monoclonal antibodies and even more are expected in the coming years. Knowledge of not only their mechanism of action but also their adverse event profi le is tantamount. One of the distinctive side effects of these drugs is the potential for non-allergic and allergic infusion reactions caused by cytokine release. These adverse reactions should be monitored and managed immediately for patient welfare and safety. General Objective : To determine the prevalence of infusion reaction among patients given monoclonal antibodies at outpatient infusion units of a University Hospital, from July 2015 to July 2017. Methodology : This is a two-year retrospective study at the University of Santo Tomas Hospital (USTH), a tertiary teaching hospital. A chart review of patients seen at the USTH Benavides Cancer Institute (BCI) and Joint and Bone Center (JBC) were gathered. Patients who received monoclonal antibodies namely rituximab, infl iximab, bevacizumab, tocilizumab, belimumab, brentuximab, pembrolizumab, trastuzumab, pertuzumab, nimotuzumab and eculizumab from July 2015 to July 2017 were included in the study. Results : Majority of patients were in the 61 to 70 years age group (25.7%); the median age of the population was 53 years and the majority were females (64.9%). The most common indication for monoclonal antibody infusion is rheumatoid arthritis (28.4%). More than one-fourth of the patients did not receive premedications (28.4%) but a good number (25.7%) received at least two drugs: paracetamol plus diphenhydramine plus corticosteroids followed by 17.6% who received paracetamol plus diphenhydramine. The authors found a signifi cant difference in the occurrence of infusion reaction between those that were given premedications compared to those who were not given premedications (p=0.032). The most common monoclonal antibodies administered were rituximab (28.4%); tocilizumab (23%); and infl iximab (14.9%). However, only rituximab (9.5%), infl iximab (2.7%) and bevacizumab (1.4%) had infusion reactions. The overall incidence rate of infusion reaction to monoclonal antibodies was 14%. The onset was within 2 hours with most infusion reactions occurring in the fi rst cycle. Ninety percent were classifi ed as grade 2 infusion reactions. Management of these reactions included rescue medications and brief interruption of infusion. No hospitalization nor recurrence of infusion reaction on the resumption of infusion occurred. Conclusions The prevalence rate of infusion reaction to monoclonal antibodies was 14% in this present single-center two-year retrospective study. All adverse events were graded mild to moderate (grade 2) in severity, and were all accordingly managed successfully in the outpatient setting.
Antibodies, Monoclonal ; Prevalence

RATIONALE AND OBJECTIVES

Individuals with myeloproliferative neoplasia (MPN) have blood cell parameters representing abnormal proliferation of the cell line/lines affected. Considering the implication of symptom burden scores to treatment response and disease progression, with the same implication among changes in blood cell parameters, a question of correlation between the two variables becomes inevitable. This study aims to determine the correlation of controlled blood counts, severity of symptoms and quality of life of individuals with MPN.

This is a cross-sectional analytical study and a sub-study from the Filipino myeloproliferative neoplasia quality of life (MPN-QOL) multicenter study. Secondary data obtained from the parent study will be used as primary data of this sub-study. Comparative analyses were conducted using Chi-Square Test of Homogeneity or Fisher’s Exact Test. Association analysis used Cramer’s V coefficient.

Data in this study has shown 52.65 years old as the average age of participants. Most participants had mild symptom burden at 60.53% with the most common symptom being fatigue. Comparative analysis showed the absence of identified statistical difference in the overall symptom burden severity among the three types of MPN.

In this study, there was no statistically significant correlation between the severity of symptom burden or quality of life, and the degree of blood count control among the three types of MPN. In practice, controlling hematologic parameters has been a goal to achieve among patients with MPN. This study suggests symptom control and quality of life is not necessarily affected by blood count control.

Introduction: Multiple myeloma remains to be an incurable hematologic entity, but with the advent of novel agents more patients experience significantly longer survival. In a third world country like the Philippines, autologous bone marrow transplant after chemotherapy for newly diagnosed cases which is the standard of care is difficult to comply. The management paradigm for myeloma has shifted over the years, hence this study.

Objective: Determine the clinical profile and treatment outcome of Filipino multiple myeloma patients diagnosed and managed at a tertiary institution from January 2013 to December 2018.

Methodology: Retrospective, observational and cross-sectional study of eligible symptomatic myeloma patients.

Results: Data for six years were retrospectively collected from a single tertiary institution. The clinical characteristics at diagnosis, treatment and survival rates of 109 active myeloma patients were described. The median age was 61 years (range, 28-83), with 51.4% being female. Median overall survival was 49.5 months (95% CI 42.7-56.2). The frontline treatments of patients were also analyzed. The combined deep response (complete and very good partial) of our patients at 31.7% was higher than of Asian Myeloma Network Study at 30.9%. None of them yet underwent autologous bone marrow transplantation as of date. Novel agents, especially bortezomib was used in 35.7% and significantly affected overall and progression-free survivals when used as a first line treatment.

Conclusion: This retrospective analysis demonstrated the paradigm shift in the treatment modality of myeloma and the survival outcomes has significantly improved, especially on the best response to chemotherapy. Short of the ideal management in a third world country like the Philippines, we can now set our new standard of care based on the treatments available including novel agents like bortezomib, and the best practices that our institution offers.

Rationale: COVID-19 is a new, rapidly emerging zoonotic infectious disease. Addressing the cytokine storm and coagulopathy associated with this disease can minimize its severity and complications. Therapeutic plasma exchange (TPE) can be potentially used to remove these deleterious cytokines and procoagulant proteins. Objective: This study aims to assess the effectiveness and safety of TPE as an adjunctive treatment for COVID-19 patients. Research Design and Methodology: A systematic search of databases was conducted utilizing PubMed and Cochrane databases to identify relevant literature until December 31, 2020. All publications were included if they use TPE in COVID- 19 patients. The exclusion was applied in publications written in language other than English, review papers, or on-going clinical trials. No restrictions on age, sex, or clinical setting were applied. The eligible studies were reviewed in full text independently by two authors. Methodological quality and risk of bias assessment were done. The findings from the individual studies were summarized. Results: A total of 21 studies were included. Overall risk of bias was high within and across the studies. All studies reported marked improvement of clinical status and laboratory results after receiving the TPE. The use of TPE among COVID-19 patients resulted in no serious or life-threatening adverse events. Conclusion The available studies on the use of TPE for COVID-19 patients is still limited and evidence is of low certainty. However, based on the available data, it has an encouraging result to be used as effective and safe adjunctive treatment in COVID-19 patients.
COVID-19 ; Cytokine Release Syndrome

Introduction: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy derived from the precursors of plasmacytoid dendritic cells. This malignancy presents with various noticeable cutaneous lesions and usually occurs in elderly males. Cutaneous manifestations usually precede leukemic dissemination to the lymph nodes, bone marrow, and peripheral blood which is associated with poor prognosis. Case presentation: We present a case of a 60-year-old Filipino male with a four-month history of multiple hyperpigmented, reddish brown, firm, fixed, non-tender cutaneous nodules on the extremities, trunk, chest, and face. Two large masses was also noted on the left arm and left upper back..Tissue biopsy of the cutaneous mass showed Immunohistochemical stain findings positive for LCA, CD68, CD4, CD56, and CD123 which are compatible with BPDCN. Patient was initially asymptomatic with relatively normal blood count and was treated supportively but serial blood count monitoring showed worsening with progression to acute myelogenous leukemia. Patient was then started on the 7+3 protocol of cytarabine and idarubicine which provided flattening of the cutaneous nodules and improvement of blood counts. However, due to complications of the disease and the treatment, the patient succumbed to severe pulmonary infection and sepsis. Discussion: Due to the varied, non-specific cutaneous manifestations and the similarity in the morphology of the skin lesions with other cutaneous conditions along with the rarity of this disease, there is difficulty in establishing the diagnosis of BPDCN as well as standardizing its treatment. Immunohistochemical stains play an important role in confirming the diagnosis as well as ruling out other differential diagnoses to tailor appropriate treatment. Conclusion Blastic plasmacytoid dendritic cell neoplasm (BPDCN) generally has a poor prognosis owing to the rapidity of its spread to the bone marrow and peripheral blood. Early diagnosis is essential to initiate early therapy and prevent progression.
Leukemia, Myeloid, Acute

BACKGROUND

Myeloproliferative neoplasms (MPN) are a heterogeneous group of disorders characterized by the cellular proliferation of one or more hematologic cell lines. Patients with MPN who are Philadelphia-negative such as those with Polycythemia Vera (PV), Essential Thrombocytosis (ET), or Myelofibrosis (MF) experience a cluster of symptoms related to the disease activity which can affect their quality of life.

This study aimed to explore the symptoms and symptom experience as well as lived experience of Filipino patients with MPN using a qualitative phenomenological approach to get a deeper understanding of the disease symptomatology.

Twenty-three patients with myeloproliferative neoplasms were purposively selected according to: 1) type of MPN (PV, ET, MF) 2) status of MPN disease (newly diagnosed vs. chronic) 3) age (≤50 years old; >50 years) and 4) sex (male vs. female). The investigators conducted key informant interviews using a semi-structured interview guide. Interview scripts and narratives were transcribed and analyzed using categorical aggregation and thematic analysis.

Twenty patients proceeded with the interview (8 PV, 6 ET, 6 MF). The meta-themes identified were 1) symptom experience and 2) disease perception. Three sub-themes under symptom experience were a) heterogenous and complex symptomatology; b) dynamic nature of symptoms; c) living and coping with symptoms. Three sub-themes under disease perception were a) struggle with the concept of the disease; b) anxiety and uncertainty; c) acceptance and hope. The most common symptoms experienced by the patients were fatigue, bone pain, and abdominal discomfort. Vascular symptoms specifically headache, numbness, and problems in concentration were commonly reported by patients with PV. Fever and weight loss were the least common. Sources of anxiety/uncertainty include the unpredictability of symptom occurrence and blood counts, the burden of taking maintenance medications, the financial burden of living with MPN, and the unpredictability of disease and complications.

Patients with MPN had heterogenous, co-occurring, and dynamic symptoms which affected their overall productivity both at home and at work. Patients with MF had the most symptom burden while patients with ET had the least. This study provided valuable insights on disease perceptions, sources of anxiety, and coping mechanisms of patients with MPN. A deeper understanding of the symptom experience and disease perceptions of the patients will enhance the physician-patient interaction especially when discussing management options.

BACKGROUND AND OBJECTIVE

Myeloproliferative disorders are characterized by symptoms that can potentially impair the quality of life of our Filipino patients. The Myeloproliferative Neoplasm Symptom Assessment Form-Total Symptom Score (MPN-SAF TSS) is used to determine symptoms at baseline and during treatment. A validated Filipino version of this questionnaire would be a helpful tool in assessing the burden of symptoms of Filipino patients with MPN. Understanding the symptom burden and symptom experience of patients with MPN is needed in developing a comprehensive wholistic management plan that addresses the physical and emotional aspects of a chronic disease such as MPN. This study aimed to translate the MPN-SAF TSS to the Filipino language.

Translation, validation, and reliability testing of the MPN-SAF TSS was done following the International Society for Pharmacoeconomics and Outcomes Research (ISPOR) guidelines for linguistic translation and validation.

No major discrepancies were found on forward and backward translation of the MPN-SAF TSS tool. The 0-10 scoring scale (where 0 = absent/as good as it can be and 10 = worst imaginable/daily/as bad as it can be) was deemed appropriate and was retained. Content and face validity as evaluated by Filipino hematologists showed that majority of the translated questions were relevant. Some words were further improved according to their valid recommendations. Ten MPN patients voluntarily answered the updated Filipino version of the questionnaire for face validity and cognitive debriefing was done to further refine the translation according to the patient’s perspective. The back translation was identical to the original versions of MPN-SAF TSS.

Validity and reliability testing of the revised and translated MPN-SAF TSS among 30 patients with MPN demonstrated that the MPN-SAF TSS Filipino version was conceptually equivalent with the English version, with good internal consistency (Cronbach’s alpha=0.89); excellent reliability with an intraclass correlation coefficient of 0.98 and convergent validity of r=0.77. 

The Filipino version of the MPN-SAF TSS was demonstrated to be a valid and reliable tool in evaluating the symptom burden of Filipino patients with MPN.