Acquired angioedema in a patient with chronic lymphocytic leukaemia

Acquired angioedema [AAE] is defined by acquired deficiency of C1 esterase inhibitor. It is a rare disorder characterized by recurrent angioedema without urticiaria, which may be associated with lymphoproliferative disorders [LPD]. We are reporting a case of 71 years old female, known case of Chronic Lymphocytic Leukaemia [CLL] who presented to the emergency room [ER] with generalized body swelling during her disease course. Investigations were ordered to confirm that the symptoms were due to acquired angioedema, and the patient was managed for this diagnosis. Knowing the association between acquired angioedema and lymphoproliferative disorder is crucial, because AAE can be treated medically and responds within hours but delayed diagnosis can lead to unnecessary invasive procedures or asphyxiation and death due to laryngeal oedema. Finally, treating the underlying cause as CLL in our case could prevent further recurrence of angioedema

Acquired angioedema in a patient with chronic lymphocytic leukaemia

Acquired angioedema [AAE] is defined by acquired deficiency of C1 esterase inhibitor. It is a rare disorder characterized by recurrent angioedema without urticiaria, which may be associated with lymphoproliferative disorders [LPD]. We are reporting a case of 71 years old female, known case of Chronic Lymphocytic Leukaemia [CLL] who presented to the emergency room [ER] with generalized body swelling during her disease course. Investigations were ordered to confirm that the symptoms were due to acquired angioedema, and the patient was managed for this diagnosis. Knowing the association between acquired angioedema and lymphoproliferative disorder is crucial, because AAE can be treated medically and responds within hours but delayed diagnosis can lead to unnecessary invasive procedures or asphyxiation and death due to laryngeal oedema. Finally, treating the underlying cause as CLL in our case could prevent further recurrence of angioedema

Establishing an autologous versus allogeneic hematopoietic cell transplant program in nations with emerging economies

More than 70,000 hematopoietic cell transplants are currently performed each year, and these continue to increase every year. However, there is a significant variation in the number of absolute transplants and transplant rates between centers, countries, and global regions. The prospect for emerging countries to develop a hematopoietic cell transplantation [HCT] program, as well as to decide on whether autologous HCT [auto-HCT] or allogeneic HCT [allo-HCT] should be established to start with, relies heavily on factors that can explain differences between these two procedures. Major factors that will influence a decision about establishing the type of HCT program are macroeconomic factors such as organization of the healthcare network, available resources and infrastructure. Prevalence of specific diseases in the region as well genetic background of donors and recipients will also influence the mandate or priority of the HCT in the national healthcare plan to explain some of the country-specific differences. Furthermore, microeconomic factors play a role, such as center-specific experience in treating various disorders requiring hematopoietic stem cell transplantation, along with accreditation status and patient volume. The objective of the transplant procedure was to improve the survival and quality of life of patients. The regional difference that one notices in emerging countries about the higher number of allo-HCT compared with auto-HCT procedures performed is primarily based on suboptimal healthcare network in treating various malignant disorders that are the primary indication for auto-stem cell transplantation. In this context, nonmalignant disorders such as bone marrow failure syndromes, inherited genetic disorders and hemoglobinopathies have become the major indication for stem cell transplantation. Better understanding of these factors will assist in establishing new transplant centers in the emerging countries to achieve their specific objectives and positive outcome

Plasma cell leukemia: clinicopathologic, immunophenotypic and cytogenetic characteristics of 4 cases

Plasma cell leukemia [PCL] is a rare hematologic malignancy with very poor outcome. It is defined by the presence of >2 · 109/L plasma cells or >20% plasmacytosis of the differential white cell count in the peripheral blood. Primary PCL is first diagnosed in the leukemic phase, while secondary PCL corresponds to the leukemic transformation of a previously diagnosed multiple myeloma [MM]. The incidence of PCL ranges between 2-4% of patients with MM and 0.9% of patients with acute leukemia. In this case series, we describe the clinicopathologic, immunophenotypic, and cytogenetic findings of four patients diagnosed with PCL within a ten-year period [2002-2012] at King Faisal Specialist Hospital and Research Centre [General Organization], Riyadh, Saudi Arabia

Maternal acute lymphocytic leukemia with rearrangement of the mixed lineage leukemia gene occurring during pregnancy

Acute lymphoblastic leukemia [ALL] is a relatively rare disease during pregnancy, accounting for about 15% of all cases of pregnancy-associated leukemia. Although mixed lineage leukemia gene [MLL] rearrangement is the dominant genetic aberration in infantile acute leukemia, the occurrence of MLL gene rearrangement in maternal ALL occurring during pregnancy has not been reported. Out of 31 cases of maternal leukemia diagnosed during pregnancy at our institution, 5 were ALL cases. Three of the 5 patients had MLL gene rearrangement. The data for these 5 patients are presented in this report. We believe that the association of MLL gene rearrangement with maternal leukemia is biologically plausible and this observation needs to be validated in a larger cohort of pregnancy-associated maternal leukemia cases