Diagnostic Evaluation of Polycythemia Vera at High Altitude.

BACKGROUND: Polycythemia is a common medical problem, frequently acquired and reactive to secondary conditions. High-altitude-associated hypoxia contributes to the greater prevalence of polycythemia at altitude. Primary clonal polycythemia vera (PV), even though it is rare, requires a different therapeutic approach. Suspicion of PV usually drives the diagnostic workup of polycythemia. METHODS: In this retrospective lab record study, we collected all JAK2 tests requested over a three-year period. We analyzed requests that were made for the evaluation of polycythemia. Complete blood count (CBC) and imaging of the abdomen were collected. RESULTS: Out of 208 total requests, 136 were for the purpose of polycythemia evaluation. JAK2 mutation was positive (confirming the presence of PV) in 22 (16.7%) cases. PV patients have the usual demographics reported elsewhere. Additionally, PV patients exhibit distinct hemogram results featuring leukocytosis, thrombocytosis, and hypochromic microcytic red blood cells (RBCs) related to the associated iron deficiency. CONCLUSIONS: Many patients with polycythemia at altitude might be unnecessarily considered for an evaluation of PV, if hemoglobin/hematocrit is the sole deciding criterion. PV patients have a distinct CBC pattern that can be exploited to better select patients with polycythemia for further evaluation and thus reduce unnecessary workups.

Overuse Ordering Serum Vitamin B12: A Call for Appropriate Ordering Guidelines Based on a Single Institutional Experience.

OBJECTIVE: Vitamin B12 is an essential nutrient for numerous functions in the human body. As such, many clinical scenarios justify assaying serum B12; however, there are no evidence-based guidelines available for determining when to order B12 tests. Here we investigate B12 assay ordering patterns to identify methods of enhancing efficiency and minimizing inappropriate requests. METHODS: All serum B12 requests within the year spanning July 2018 to June 2019 were reviewed using the hospital's health information system. These amounted to 3,400 requests, of which data from the first 1,000 were evaluated. Patient demographics, hematological data, serum folate, and ferritin were extracted from the electronic requests. Physician identity and request reason were retrieved where available. RESULTS: Of the 877 B12 requests for which patient age was available, the majority (80.3%) were for young and middle-aged patients. Interestingly, serum B12 was low in only a quarter of the 1,000 reviewed requests; the remaining three quarters had normal levels. Folate, which can be ordered with vitamin B12, was tested in 82 cases, of which only two (2.4%) had folate deficiency. CONCLUSION: This study highlights a high occurrence of improper ordering of vitamin B12 assays, indicating a need for revised guidelines to promote optimal test ordering.

Idiopathic Thrombocytosis in Alpha Thalassemia Trait Patient.

Platelet count increases are typically a reactionary response to one of a variety of pathophysiological events. We present here a case of microcytic hypochromic red blood cells and thrombocytosis in an adolescent female that we have monitored for three years. The patient was positive for alpha thalassemia trait; negative for mutation in Janus kinase 2, calreticulin, or myeloproliferative leukemia virus oncogene; and negative for reactive causes of thrombocytosis. Noticeably, a variant in atypical chemokine receptor 1 (ACKR1) (c.-67T>C, rs2814778) was found to be homozygous. Accordingly, the case was diagnosed as idiopathic thrombocytosis, and treatment was given to restore platelet levels to normal. Our findings highlight the possibility of an unknown association between alpha thalassemia trait and idiopathic thrombocytosis in the presence of ACKR1 mutation, which could be implicated in disease pathogenesis.

The attitude of the Public Toward Social History Taking in Southern Saudi Arabia.

Background: Social history taking is an important element of a medical interview. No previous studies, however, addressed public attitudes regarding social history taking in Saudi Arabia. Objective: This study explores public attitudes about the importance of social history taking by doctors in Southern Saudi Arabia. Methods: A cross-sectional study was conducted to collect public opinion about social history taking using a self-administered questionnaire. Social history taking was defined as questions about general sociodemographics (job, income, marital status, housing conditions, and animal contact) and social habits (travel, smoking, alcohol drinking, illicit drug use, and sexual history). Descriptive statistics were conducted, along with a comparison between categorical variables. Results: A total of 510 respondents completed the questionnaire, of which 381 (74.7%) had a medical history taken before. A complete social taking was not obtained from those with medical history previously taken. Marital status (73.2%), smoking (49.1%), and occupation (32.8%) were the commonest inquiries, while income (3.7%) and sexual history (6.6%) were the least. Most respondents had no reservations about answering questions about their social history. Among the general demographic questions, job and marital status were more likely to be answered, whereas income and housing inquiries were less likely to be answered. Among social habits, sexual history followed by illicit drug use and alcohol drinking inquiries were less likely to be answered. Conclusion: Detailed social history taking is uncommon among doctors. The public is open to discussing their social status and habits. However, questions involving income, housing, and some social habits like illicit drugs, alcohol, and sexual history are less likely to be faithfully reported and thus need to be handled appropriately. It is crucial for healthcare providers to approach social history taking with sensitivity and respect and to create a private environment for patients where they can freely and accurately share their social histories.

Effect of Altitude on Hemoglobin and Red Blood Cell Indices in Adults in Different Regions of Saudi Arabia.

Purpose: Complete blood count (CBC) is a commonly used blood test for health checks. This study was conducted to compare CBC from two different altitudes and from sea-level populations in order to suggest correction factor for altitude-related increment on the hemoglobin and red blood cell indices. Patients and Methods: In this retrospective lab records study, large datasets of CBCs over 5-year period were screened from three different regions of Saudi Arabia, Jeddah (a coastal city), Taif City located at 1879 m above sea level, and Abha City at located 2270 m above sea level. Descriptive analysis and significance testing of the CBC variables at different altitude were compared. Results: A total of 57,059 participants were included for final analysis. Mean hemoglobin (Hb) concentration (g/dL) was 14.81 for men and 13.77 for women at sea level, whereas Hb concentrations were 15.35 and 14.19 for men and women residing at Taif City, respectively, and 15.40 and 14.71 for men and women at Abha City, respectively. Hemoglobin and other red cell indices were significantly different among men and women across different altitude, except for mean corpuscular volume (MCV). The MCV 95th percentile range was lower at sea level and both altitudes as compared to current reference range (76-91 fL vs 81-98 fL). Conclusion: Although altitude-associated increment in Hb concentration was evident in both men and women, it was lower than as suggested by World Health Organization and Centers for Disease and Control. Results of this study can also be used to derive new CBC reference intervals for Saudi residents living at high altitude. A correction factor (ΔHb) of 0.30 g/dL per 1000 m altitude is suggested to be used in adult population living at high altitudes in Saudi Arabia which should help better define anemia and polycythemia at altitude.

Isolated Neutropenia/Benign Ethnic Neutropenia: A Common Clinical and Laboratory Finding in Southern and Western Saudi Arabia.

OBJECTIVE: Isolated mild neutropenia is a common clinical problem in some ethnicities including Arabs and Middle Eastern population. The current study aims to authenticate the prevalence of isolated neutropenia in Southern and Southwestern Saudi Arabia, explore the effect of altitude or regional differences and to suggest a new reference range for neutrophil count. METHODS: In this retrospective cross-sectional study, laboratory results of a commercial laboratory were screened over a period of 5 years (2016-2020) in seven different cities of different altitudes in South and southwestern Saudi Arabia. Participants' laboratory investigations were reviewed and excluded for any abnormal complete blood count, renal profile, liver profile, lipid profile, thyroid function test, fasting blood glucose, or HbA1c findings. Descriptive analysis and 95th percentile range were calculated using standard statistical methods. RESULTS: A total of 91,880 complete blood count results were included in the final analysis. Isolated neutropenia was common laboratory finding, with a prevalence ranging from 11% to 23%. The 2.5th percentile of the neutrophil count was lower than currently utilized 1.5×109/L in all studied seven cities. CONCLUSION: Mild to moderate neutropenia is common in Southern and Southwestern Saudi Arabia. Benign ethnic neutropenia (BEN) likely explains this high prevalence. Since BEN has no clinical significance, the reference range for normal neutrophil counts needs to be adjusted to reflect the effect of BEN.

The Prevalence of Isolated Neutropenia at High Altitude in Southern Saudi Arabia: Does Altitude Affect Leucocyte Count?

INTRODUCTION: Isolated neutropenia is a common problem in hematology practice. Benign ethnic neutropenia (BEN) is the common form of neutropenia worldwide and it affects mainly African and Middle Eastern ethnicities. Most cases of isolated mild and moderate neutropenia are benign and associated with no clinical significance. The aim of this study was to estimate the prevalence of isolated neutropenia at high-altitude southern Saudi Arabia. METHODS: A large dataset of complete blood counts (CBCs) was analyzed for walk-in patients of both genders, of age ranges from 12 to 60 years doing blood tests at a commercial lab for high altitude - 2,270 meters above sea level - (HA) group, and from sea level SL group. Abnormal biochemical or CBC results were excluded before analysis. RESULTS: For HA group, 3123 CBCs were analyzed and for SL group 18,427 CBCs were analyzed. The prevalence of mild neutropenia, defined as absolute neutrophil count (ANC) in the range of 1.0-.5× 109/L, was 12.4% (n=399) versus 5.6% (n=1025), while moderate neutropenia, defined as ANC of 0.5-1 × 109/L, was present 6% (n=191) versus 1.45% (n=269) in the high altitude and sea level groups, respectively. Severe neutropenia, defined as neutrophil count less than 0.5-1 × 109/L, was rare in both groups. CONCLUSION: Isolated neutropenia is common in Saudis living at high altitude. While benign ethnic neutropenia (BEN) is thought to be the major contributing factor to this high prevalence, other factors including environmental factors and altitude are possible contributing factors along with underlying ethnic neutropenia.

The pattern of iron deficiency with and without anemia among medical college girl students in high altitude southern Saudi Arabia.

INTRODUCTION: The prevalence of iron deficiency, latent and symptomatic, is heterogeneous worldwide. In this study, we aim to explore the prevalence of iron deficiency, with and without anemia, among medical college females at the high Altitude Aseer region of Southwestern Saudi Arabia. METHODOLOGY: 200 female medical students were randomly sampled, between the ages of 19 and 27 years. Blood samples were collected for complete blood count (CBC) values and serum ferritin determination. Questionnaires were completed in order to collect demographics, medical history, and socioeconomic information of the participants. RESULTS: Prevalence of overall iron deficiency was high (63%, serum ferritin <20 µg/L, 52.5%, ferritin <15 µg/L). Anemia, adjusted for high altitude (defined as less than 13 g/dL was present in 41 participants (20.5%) and 12 (6.5%) have Hb <12 g/dL. Iron-deficiency anemia (Hb <13 g/dL and serum ferritin <15 µg/L) was present in 35 (17.5%). Personal and family history of anemia and poor animal product containing meals were positively correlated with the presence of iron deficiency state. Neither symptoms of anemia, nor the presence of menorrhagia correlate with the presence of iron deficiency anemia. CONCLUSION: Iron deficiency with and without anemia is a very common and condition in young females' population at high altitude. Implementing a lab method to screen for anemia on vulnerable populations is needed. Frequently asymptomatic, the primary care providers should maintain a high degree of suspicion in order to initiate screening for iron status.

Isotretinoin is active in the initial management of acute pro-myelocytic leukemia.

Pro-myelocytic acute leukemia (APL) is characterized by the proliferation of cells blocked at promyelocytic stage and ATRA is the choice of initial treatment because of the APL sensitivity to this compound. In this case study we report a 28-year-old man who presented to the Emergency Department with epistaxis, petechial rash, and fever. Laboratory tests revealed the presence of high white blood cell count with 60% blasts and evidence of coagulopathy. The diagnosis was confirmed later as APL. Because of the delayed transfer to the reference center and unavailability of ATRA initial treatment, the patient received isotretinoin, a related compound. The treatment was successfully implemented in the initial management of acute pro-myelocytic leukemia as patient condition improved. isotretinoin could be used as an alternative therapy for ATRA whenever the latter is not available. further research is needed to establish the appropriate doses and to assess the potential risk of differentiation syndromes.

Clinical Appropriateness of Serum Folate ordering pattern in a tertiary care hospital in Saudi Arabia.

Folate, also known as vitamin B9, is a co-factor necessary for DNA synthesis. Folate deficiency is associated mainly with hematological findings including megaloblastic anemia and pancytopenia. Many countries have mandated grain fortification with micronutrients including folic acid resulting in a reduced prevalence of folate deficiency. Saudi Arabia imports most of the grain products and folate is usually added after milling. There are no local studies to address the folate deficiency prevalence. In this study we aimed to analyse the clinical appropriateness of ordering practice of serum Folate level. METHOD: We reviewed all serum folate requests received at our laboratory in Aseer Central Hospital over one-year period (July 2018 June 2019). We collected patients' demographics from the electronic requests along with biochemical results of serum B12, ferritin and CBC results. We assessed appropriateness of orders against pre-specified criteria and applied statistical tests to explore for any association or significance. RESULTS: Serum folate requests from 614 patients were received during the study period. Serum B12 (543, 88%), and serum ferritin (511, 83%) were concurrently requested. The most common reason for request, when available, was anemia. Anemia was present in (313, 51%) of the subjects for which microcytic anemia was predominant (199, 63.5%), followed by normocytic anemia (101, 33%) and only 10 subjects had macrocytic anemia (3.2%). The most common hematinics' deficiency was ferritin (30%) followed by B12 (17.2%). Serum folate deficiency was low, observed in only 2.8%. Low folate levels were not significantly different between the group with anemia and the normal hemoglobin group. CONCLUSION: This study identifies a commonly inappropriate serum folate ordering practice that includes ordering all hematinics at the same visit without considering the possible anemia etiologies. The excessive requests might be related to doctors attempt to avoid multiple blood extractions and to try to reduce the time for diagnosis. These policies are generating unnecessary costs and time loss. Education, phasing out or restricting some tests and introducing laboratory policies like sample storing could help reduce unnecessary requests.