Prevalence of neutropenia among adult Arabs in Qatar: Relation to other hematological parameters and anthropometric data.

Neutropenia ranges from a normal variant to life-threatening acquired and congenital disorders. This study aims at providing baseline information regarding the prevalence and spectrum of neutropenia in the Arab blood donors who are living in Qatar. This retrospective cohort study was conducted to review the data of healthy Arab individuals (≥18 years) who donated blood between January 1, 2015 to May 15, 2019. A complete blood count was performed using automated analyzers. The prevalence of neutropenia was 10.7%. The prevalence in females was 32% and in males, it was 6%. Absolute neutrophil count (ANC) below 1 × 109/L was detected in 10% of Arab females and 1.8 % of Arab males. In females, the neutropenic group had significantly lower hemoglobin (Hb) levels and higher red cell distribution width, and lower total white blood cells and lymphocyte counts (P < .001) compared to the group with ANC > 1.5 × 109/L. Significant correlations were found between the ANC and Hb (r = 0.33, P < .05) and ANC and total white blood cells (r = 0.45, P < .01). The prevalence of neutropenia is considerably high in Arab adult females compared to other ethnic groups. Besides the genetic constitution of Arabs, the lower Hb and higher red cell distribution width in females suggest that iron deficiency could contribute to the development of neutropenia.

Hematological indices reference intervals for a healthy Arab population in Qatar: Effect of age, gender, and geographic location.

ABSTRACT: Hematologic reference intervals vary with gender, age, ethnicity, and geographic area. Therefore, local or national laboratory reference ranges are essential to enhance the accuracy when diagnosing health conditions. Still, no comprehensive list of reference ranges tailored to the Arab population living in Qatar. Accordingly, this study aims at establishing a hematology reference guide for Arabs in Qatar.This is a retrospective study where 750 healthy volunteers (18-69 years) from 2015 to 2019 were included, analyzed by an automated hematology analyzer. Arab adults were divided into African (Egypt, Libya, Tunisia, Morocco) and Asian (Syria, Lebanon, Jordon, Palestine, Qatar). The Cell-Dyn and Sysmex were used for measuring hematological parameters.The mean +/- 2SD were established for all the study groups. Arab males had significantly higher Hb, Hct, red cell distribution width, absolute neutrophil count, lymphocytes, and monocyte counts than females. Asian-Arab males had significantly higher Hb concentration and higher WBC, lymphocytes, and eosinophils than African Arabs. Asian-Arab young (>18: < 40 years) males had significantly higher Hb and lymphocytes and lower monocytes than older males (>40 years). African-Arab young males had significantly higher lymphocytes and lower monocytes than older males. Asian-Arab young females had higher WBC and absolute neutrophil count than older Asian Arabs.The findings of this study will help in establishing specific reference intervals in the Arab world. The differences in hematology reference intervals considering age, gender, and geographical location highlight the importance of establishing blood reference intervals in each country considering the ethnic diversity of each country.

Clinicopathological Variables and Outcome in Chronic Myeloid Leukemia Associated With BCR-ABL1 Transcript Type and Body Weight: An Outcome of European LeukemiaNet Project.

OBJECTIVE: It is debatable whether BCR-ABL1 transcript type has an impact on outcome of treatment of patients with CML, and it is not widely studied whether body weight influences response to treatment. In this study, we tried to find out if any of these factors has an impact on response to treatment and outcome. METHODOLOGY: We conducted a retrospective analysis of the files of 79 patients being treated in our center for CML with known BCR-ABL1 breakpoints, and patients' management and response assessment was done based on ELN 2013 guidelines. The analysis was performed based on two main groups, obese vs. normal BMI, and then based on BCR-ABL1 transcripts: e13a2 vs. e14a2. Cumulative incidence of MMR, CCyR, and DMR were estimated using the Kaplan-Meier survival curve method, and comparisons between groups were performed by the Log-rank/Gray test methods. RESULTS/CONCLUSION: In the patient-cohort studied, there was no statistically significant difference in molecular response between patients with CML based on body weight or transcript type although patients in the obesity group achieved higher and faster MMR with no statistical significance.

Chronic Myeloid Leukemia Preceded by Tuberculosis.

Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm, classically described as a triphasic disease. However, little is known about risk factors for developing CML. Currently, ionizing radiation is the only established risk factor. Here, we report on a 37-year-old man treated for tuberculosis; 2 years later, he developed CML in a chronic phase. We would like to shed light on tuberculosis as a possible risk factor for CML.

A Rare Case of Systemic Mastocytosis with Associated Hematologic Neoplasm (SM-AHN) Involving Chronic Myeloid Leukemia: A Case Report and Literature Review.

BACKGROUND Single or multiple cell line dysplasia is a characteristic feature of myelodysplastic syndrome. However, significant dysgranulopoiesis is not a feature of chronic myeloid leukemia (CML). Systemic mastocytosis (SM) with an associated hematologic neoplasm (SM-AHN) comprises 5% to 40% of cases of SM. All types of hematologic neoplasms have been previously reported, although CML has been rarely encountered. CASE REPORT A 28-year-old male presented with a 3-month-history of weight loss and massive splenomegaly. Peripheral blood revealed marked leukocytosis, shift to left with 13% blasts. There was evident dysgranulopoiesis that raised a provisional diagnosis of myelodysplastic/myeloproliferative neoplasm. Bone marrow (BM) examination revealed granulocytic hyperplasia with 10% blasts and significant dysgranulopoiesis. Unexpectedly, cytogenetic analysis revealed t(9;22) with BCR/ABL1 rearrangement, diagnostic of chronic myeloid leukemia in an accelerated phase. The patient was started on dasatinib 100 mg upfront, however, he failed to respond, with increasing leukocytosis. Repeat BM examination showed persistence of the findings with 8% blasts. At this time, aggregates of mast cells with aberrant expression of CD25 were elicited, thus concluding the diagnosis of SM-AHN. The patient failed multiple lines of treatment (dasatinib, nilotinib, hydroxyurea, cytarabine subcutaneous, 6-mercaptopurine and interferon) and progressed to the blast phase a few months later. CONCLUSIONS We report an unusual case of CML, presented with significant dysgranulopoiesis with an aggressive clinical course including SM uncovered during the disease course with subsequent transformation to the blast phase. The different biological behavior of this case underscores the need for studies on a larger number of cases to explore the significance of the aforementioned coexistent features.

Subcutaneous Masses as an Unusual Manifestation of Relapse in a Case of Atypical Chronic Lymphocytic Leukemia with Prolymphocytoid Transformation and Complex Karyotype: A Diagnostic Dilemma and Treatment Challenge.

BACKGROUND Most patients with chronic lymphocytic leukemia (CLL) are asymptomatic at diagnosis, but 10% present with B symptoms. Most patients have palpable lymphadenopathy, while 20-50% of the patients have hepatosplenomegaly. Cutaneous infiltrations in patients with CLL can be localized or generalized in the form of erythematous papules, plaques, nodules and, ulceration, which is uncommon. CASE REPORT We present the case of a 71-year-old man diagnosed with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) with atypical immunophenotype and increased prolymphocytes (CLL/PLL), which was treated initially after white blood counts (WBC) doubling with Bendamustine and Rituximab for 6 cycles, and achieved complete remission. The patient relapsed after 6 months of completion of treatment, with multiple large subcutaneous masses, proved to be infiltration with the same atypical CLL/SLL on tissue biopsy, with pathologic features indicating disease progression. The lack of similar reported cases, and the aggressiveness of the tumor clinically and histopathologically, resulted in the decision to treat with Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisolone (R-CHOP) as a case of aggressive lymphoma, with complete remission clinically and radiologically. CONCLUSIONS We present a rare case of subcutaneous extramedullary masses of atypical CLL/SLL. The high proliferation index (Ki-67) and the increase of large cells are suggestive of aggressive progression of the disease; however, no frank features of Richter's transformation were noted. Based on this and because of the unusual aggressive-looking skin masses, the panel decided to treat the patient with R-CHOP. The impact of this presentation on the prognosis of the disease is not clear. To date, our patient has responded well to treatment with R-CHOP, with complete remission of the subcutaneous masses and on PET scan, but further follow-up is needed.

The Impact of Iron Overload in Patients with Acute Leukemia and Myelodysplastic Syndrome on Hepatic and Endocrine functions.

Patients with hematologic malignancies undergoing chemotherapy and requiring blood transfusion usually have an elevated serum ferritin. These findings have led to the suggestion that iron overload is common and may have deleterious effects in these patients. However, the relationship between serum ferritin and parenchymal iron overload in such patients is unknown. Therefore, we measured the liver iron content (LIC) by the FerriScan® method and investigated the liver function and some endocrine tests in 27 patients with acute leukemia (AL) or myelodysplastic syndromes (MDS). Using FerriScan® method, the normal mean LIC levels are: 4.3 ± 2.9 mg Fe/g dry weight (d.w.). In our patients, the mean serum ferritin level was 1965 ± 2428 ng/mL. In our patients, the mean total iron in the blood received by them was 7177 ± 5009 mg. In 6 out of 27 patients LIC was > 7 mg Fe/g d.w. and in 11/27 serum ferritin was > 1000 ng/ml. Measuring fasting blood glucose revealed 3/27 with diabetes mellitus and 4/27 with impaired fasting glucose (IFG). All patients had normal serum concentrations of calcium, parathormone (PTH), free thyroxine (FT4) and thyrotropin (TSH). Four patients had elevated serum alanine transferase (ALT). LIC was correlated significantly with ferritin level (r = 0.5666; P < 0.001) and the cumulative amount of iron in the transfused blood (r = 0.523; P <0.001). LIC was correlated significantly with ALT (r = 0.277; P = 0.04) and fasting blood glucose (FBG) was correlated significantly with the amount of iron transfused (r = 0.52, p < 0.01) and ALT level (r = 0.44; P< 0.01). The age of patients did not correlate with LIC, FBG or ALT. In conclusions, these results contribute to our understanding of the prevalence of dysglycemia and hepatic dysfunction in relation to parenchymal iron overload in patients with hematologic malignancies undergoing chemotherapy and requiring blood transfusions.

A persistent cough as atypical clinical presentation of intrathoracic extramedullary hematopoiesis (EMH) in a female with thalassemia intermedia.

Extramedullary hematopoiesis (EMH) is a rare disorder, defined as the appearance of hematopoietic elements outside the bone marrow or peripheral blood. The most common sites of EMH are liver and spleen, but it has been documented in other organs such as the mediastinum, lymph nodes, breast, and central nervous system. EMH occurs as a compensatory mechanism for bone marrow dysfunction in severe thalassemia. We report a case of EMH presenting as a posterior mediastinal mass in a 34-year-old woman with thalassemia intermedia with chronic cough and shortness of breath on exertion. The diagnosis of EMH was confirmed by a CT-guided fine needle biopsy. All symptoms disappeared after surgical removal of the mass.