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For over four decades, efforts have been underway for the evaluation of sickle cell retinopathy (SCR) in an attempt to identify peripheral high-risk vascular abnormalities based on Goldberg's classification (gold-standard) (1971). The macula is an area in the center of the retina that is responsible for high-resolution central vision and is also affected in SCR. With the development of new technologies for retinal imaging, the macula became a main focus of interest in the study of sickle cell disease (SCD). Optical coherence tomography angiography is a modern non-invasive method that allows qualitative and quantitative analysis of the retinal vascular network in the macular region. We identified 86 studies on SCD and optical coherence tomography angiography. Of those, 12 met the inclusion criteria for this systematic review. The prevalence of sickle cell maculopathy was 45.6% in the SCD population and it increased with age. We present the main vascular findings and discuss visual impairment and pathophysiology, genotypes, and peripheral high-risk vascular abnormalities. The role of the hematologist, general practitioner, and pediatrician in treatment is also reviewed.

The ongoing COVID-19 pandemic is the most trending and talked topic across the World. From its point of origin in Wuhan, China to clinical laboratory at NIH, a mere six-month-old SARS-CoV-2 virus is keeping the clinicians, and scientists busy at various fronts. However, COVID-19 is an emerging and evolving disease and each day brings in more data, new figures, and findings from the field of clinical practice. The role of hematologists has been increasingly recognized during the current pandemic because of several reasons. Most important of them are the characteristic hematological findings of COVID-19 patients that also have prognostic implications and that were not seen in other viral infections. The treatment of hematological complications in COVID-19 patients is very challenging given the critical care setting. There are interim and limited guidelines thus far due to the novelty of the disease. As this remains to be a quite fluid situation, all the appropriate medical societies including the major hematology bodies are proposing initial and interim guidelines (e.g. ASH guideline). This puts a hematologist on consult service in a dubious position where, he/she must tailor the recommendations on case to case basis. The purpose of this review is to provide the background context about the impact of COVID-19 on the blood system and to summarize the current interim guidelines to manage the associated hematological issues in COVID-19 infection.

There is a dire need to develop an algorithm to improve the recognition of acquired hemophilia A and B (AHA and AHB) in clinical practice. Initial and intensive care unit (ICU) management of the disorder is particular and represents a challenge for the internist/hematologist and the ICU physician. A delay in the proper treatment of bleeding episodes can lead to a life-threatening event. Expert advice should be sought as soon as possible. Succesful resolution involves accurate diagnosis, bleeding control with hemostatic and immunotherapy, and eradication of the autoantibodies to improve overall survival. Current treatment guidelines are based on the literature in the form of cases and observational studies due to a lack of randomized controlled trials. AH can be triggered by many pathologies, presenting as a paraneoplastic syndrome in case of malignancies or as surgical associated acquired hemophilia (SAHA). We have reviewed the literature from 2015 to 2021 regarding the new case reports to further assess if there is an improvement in the clinical approach.

Thrombocytopenia during pregnancy presents unique challenges for the hematologist. Obstetricians generally manage many of the pregnancy-specific etiologies, ranging from the benign (gestational thrombocytopenia) to the life-threatening (preeclampsia; hemolysis, elevated liver enzymes and low platelets syndrome; and acute fatty liver of pregnancy). However, hematologists may be consulted for atypical and severe presentations and to help manage non-pregnancy specific etiologies, including immune thrombocytopenia, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome and antiphospholipid syndrome, among others, in which maternal and fetal risks must be considered. This review provides a general approach to the diagnosis and management of thrombocytopenia in pregnancy for the consulting hematologist.

Cutaneous manifestations of myeloid malignancies are common and have a broad range of presentations. These skin findings are classified as specific, due to direct infiltration by malignant hematopoietic cells, or non-specific. Early recognition and diagnosis can have significant clinical implications, as skin manifestations may be the first indication of underlying hematologic malignancy, can reflect the immune status and stage of disease, and cutaneous reactions may occur from conventional and targeted agents used to treat myeloid disease. In addition, infections with cutaneous involvement are common in immunocompromised patients with myeloid disease. Given the varying presentations, dermatologic findings associated with myeloid malignancies can pose diagnostic challenges for hematologists and dermatologists. In this clinical review intended for the practicing hematologist/oncologist, we discuss the presentation, diagnosis, treatment, and prognostic value of the most common cutaneous manifestations associated with myeloid malignancies using illustrative macro- and microscopic figures and with a special emphasis on practical considerations.

Waldenstrom macroglobulinemia (WM) is a distinct hematologic malignancy characterized by a lymphoplasmacytic bone marrow infiltration and the presence of immunoglobulin (Ig)M monoclonal protein. Patients typically present at an advanced age, and a substantial proportion are asymptomatic at diagnosis. A unifying diagnosis of WM may be missed by an unsuspecting hematologist, as symptomatic patients present with a multitude of non-specific manifestations. Although constitutional and neuropathy-related symptoms predominate, concomitant IgM-induced hyperviscosity-associated features can provide useful diagnostic clues. There are specific indications for initiation of therapy. This review focuses on the most up-to-date management strategies of WM, in addition to highlighting the recent discoveries of MYD88 and CXCR4 mutations that have shed unprecedented light on the complex signaling pathways, and opened avenues for novel therapeutic targeting. Although WM remains incurable, with the rapid emergence and integration of effective novel therapies, its clinical course appears poised to improve in the foreseeable future.

In 1951, the young hematologist in training, Dr. William Harrington, infused himself with plasma from a patient with immune thrombocytopenic purpura (ITP). He rapidly developed severe, but transient, thrombocytopenia and was at risk for serious hemorrhage. Thus, the humoral autoimmune cause of ITP was established. Since 1953, when Dr. Harrington's in vivo studies ended, in vitro investigations have aimed to determine the molecular and cellular details of immune-mediated platelet destruction.