Suggested Guidelines for the Treatment of Mycosis Fungoides in Countries with Limited Resources.

The treatment options for mycosis fungoides (MF) have been expanding but unfortunately many of the currently used treatment modalities are unavailable in Egypt and other African/Arab countries. In addition, there is a lack of consensus on the treatment of hypopigmented MF (HMF), which is a frequently encountered variant in our population. We aimed to develop regional treatment guidelines based on the international guidelines but modified to encompass the restricted treatment availability and our institutional experience. Special attention was also given to studies conducted on patients with skin phototype (III-IV). Treatment algorithm was formulated at Ain-Shams cutaneous lymphoma clinic through the collaboration of dermatologists, haematologists, and oncologists. Level of evidence is specified for each treatment option. For HMF, phototherapy is recommended as a first line treatment, while low-dose methotrexate is considered a second line. For early classical MF, we recommend Psoralen-ultraviolet A (PUVA), which is a well-tolerated treatment option in dark phenotype. Addition of either retinoic acid receptor (RAR) agonist and/or methotrexate is recommended as a second line. Total skin electron beam (TSEB) is considered a third-line option. For advanced stage, PUVA plus RAR agonist and/or methotrexate is recommended as first line, TSEB or monochemotherapy is considered a second line option. Polychemotherapy is regarded as a final option. All patients with complete response (CR) enter a maintenance and follow-up schedule. We suggest a practical algorithm for the treatment of MF for patients with dark phenotype living in countries with limited resources.

Liver disease during and after hematopoietic stem cell transplantation in adults: a single-center Egyptian experience.

BACKGROUND: Hepatic complications are a well-known cause of both early and late mortality and morbidity in hematopoietic stem cell transplant (HSCT) recipients. Early diagnosis and management of hepatic complications is important in order to commence appropriate therapy. Conditioning regimens, acute and chronic graft versus host disease, sinusoidal obstruction syndrome, and infections among others represent major hepatic complications for the transplant recipient. We assessed liver function tests, viral markers, polymerase chain reaction, abdominal ultrasound, portal, and hepatic venous duplex in 88 patients underwent autologous and 102 patients underwent allogeneic transplant as well as liver biopsy in selected patients in this retrospective study and evaluated early and late hepatic complications and their impact on transplant outcome. RESULTS: The major cause of hepatic injury in allogeneic patients is the conditioning regimen (38.8%) followed by acute GVHD (14.7%), after day +100 chronic hepatic GVHD is the primary cause of liver injury which occurred in about 40% of allogeneic patients. In autologous patients, the first cause of hepatotoxicity is also conditioning regimen involving 27.9% of patients followed by flare of viral hepatitis in 7.9% and sepsis in 6.3% of cases. The prevalence of HCV, HBV, and CMV is 19%, 16%, and 8%, respectively. CONCLUSION: In our study, conditioning regimens, acute and chronic hepatic GVHD are frequent causes of hepatic injury following allogeneic HSCT while conditioning regimens, flare of viral hepatitis, and sepsis represent the most common causes of hepatic injury following autologous HSCT.

Combined Immature Platelet Fraction and Schistocyte Count to Differentiate Pregnancy-Associated Thrombotic Thrombocytopenic Purpura from Severe Preeclampsia/Haemolysis, Elevated Liver Enzymes, and Low Platelet Syndrome (SPE/HELLP).

The occurrence of thrombotic microangiopathy (TMA) in pregnancy is an unfortunate emergency condition. Proper diagnosis is mandatory which requires the consideration of two overlapping diagnoses: severe preeclampsia/haemolysis, elevated liver enzymes, and low platelet syndrome (SPE/HELLP) and thrombotic thrombocytopenic purpura (TTP). The long turn-around times of ADAMTS13 testing precludes the timely distinction between the two conditions. We aimed at evaluating schistocyte counts and immature platelet fraction (IPF%), as both increase in TMAs, to discriminate between TTP and SPE/HELLP of pregnancy. IPF% was measured using Sysmex XE-2100 automated hematology analyzer, and schistocyte counts were estimated microscopically as per the International Council for Standardization in Hematology-Schistocyte Working Group guidelines. The study included 30 pregnant patients with SPE/HELLP, 13 pregnant patients with TTP, and 30 women with normal pregnancy. The discrimination between the two patient categories was based on clinical judgment and TTP cases were identified using the PLASMIC score. TTP patients had higher values of IPF% than SPE/HELLP [19.5% (16.9-27.1) vs 13% (9.5-23.25); p < 0.001]; similar results were revealed regarding schistocyte counts [6.5% (3.9-8.6) vs 2.1% (1.6-3.5); p < 0.001]. IPF% and schistocyte counts were able to discriminate between TMA patients and normal pregnant women, and between and SPE/HELLP and TTP patients. Moreover, the discriminatory function of each was improved when the two parameters were used in combination. IPF% analysis should be used in conjunction with manual schistocyte counting in TMA cases to distinguish TTP pregnant patients from patients having SPE/HELLP.