Non-cryopreserved peripheral blood stem cells autologous transplantation in multiple myeloma: Bicentric study.

OBJECTIVES: The objective of this study is to evaluate the toxicity of autologous transplantation of non-frozen peripheral blood stem cells in Moroccan patients with multiple myeloma. MATERIAL AND METHODS: This was a bicentric retrospective study conducted in the Clinical Haematology Department of Mohammed V Military Teaching Hospital and at the Al Madina Clinic in Casablanca. The study period was from January 2015 to June 2019. All patients with multiple myeloma who had undergone an autologous peripheral stem cell transplant without freezing were included. Mobilisation was performed with lenograstim alone and the collected stem cells were stored for 24-48hours in a blood bank refrigerator at a temperature of 4°C. After standard conditioning with high-dose melphalan, the peripheral blood stem cells were reinjected 24 h following conditioning. RESULTS: Over the study period, 55 patients received an autologous transplant using non-frozen peripheral blood stem cells. The median richness of the CD34 cells collected was 4.5×106 CD34/kg (range: 2-12.2). The time required for neutrophil recovery was 12 days (range: 7-19). The time required for platelet recovery was 14 days (range: 9-32). The mortality rate within 100 days post-transplant was 3.6%. We did not observe any cases of graft failure. CONCLUSION: Our study finds good feasibility and low toxicity of autologous peripheral stem cell transplantation without freezing in patients with multiple myeloma.

[Thrombo-haemorrhagic disease-related hypoprothrombinemia-lupus anticoagulant syndrome revealing a light chains multiple myeloma]. / Un tableau thrombo-hémorragique en rapport avec un syndrome hypoprothrombinémie ­ anticoagulant lupique révélant un myélome multiple à chaînes légères.

Thrombosis and hemorrhage are two opposing manifestations of multiple myeloma. These hemostatic disorders are present in less than 12% of patients at diagnosis and involve various pathophysiological mechanisms. We report the case of a 39-year-old patient with multiple myeloma revealed by the association of a hemorrhagic syndrome and deep vein thrombosis related to a hypoprothrombinemia-anticoagulant lupus syndrome.

[Pyoderma gangrenosum following hematopoietic stem cell transplantation]. / Pyoderma gangrenosum après allogreffe de cellules souches hématopoïétiques.

BACKGROUND: Pyoderma gangrenosum (PG) is a rare form of neutrophilic dermatosis and is a potential complication in a number of systemic diseases. These include blood diseases, which represent 3.5% of cases, with the main forms being monoclonal gammopathy and acute myeloid leukemia. PATIENTS AND METHODS: Herein we report a case of pyoderma gangrenosum in a female patient who had undergone haematopoietic stem cell allograft six months earlier as part of her treatment for acute T-cell leukemia. DISCUSSION: This condition forms one of the general disorders potentially associated with PG and is a dermatological disorder that can occur in marrow graft patients.

CALR gene mutational profile in myeloproliferative neoplasms with non-mutated JAK2 in Moroccan patients: A case series and germline in-frame deletion.

BACKGROUND: The discovery of somatic mutations within the gene encoding calreticulin (CALR) in 2013 represented a major milestone in the molecular diagnosis of BCR-ABL negative myeloproliferative neoplasms (MPN). In fact, exome sequencing revealed that most patients with essential thrombocythemia (ET) or primary myelofibrosis (PMF) lacking JAK2 or MPL mutations, harbor somatic insertion and/or deletion in exon 9 of CALR gene. In this study, we identified the first CALR gene mutational landscape in Moroccan patients with MPN nonmutated for the JAK2 gene. METHODS: We performed Sanger sequencing of exon 9 of CALR gene in blood samples obtained from 33 Moroccan patients with ET or PMF non-mutated for JAK2. RESULTS: Of the 33 patients analyzed, we detected eight distinct variants in 15 patients (45.4%); six indel mutations, five with type 1 recurrent 52bp deletion, four with type 2 recurrent 5bp insertion and one in frame deletion which was found to be a germline variant suggesting a very rare condition in MPN. CONCLUSION: This is the first cohort reported in CALR gene mutation analysis in Morocco. Our results were concordant with studies reported up to date and very encouraging in promoting the molecular diagnosis of myeloproliferative neoplasms in Moroccan patients. Moreover, the presence of a germline in frame deletion in a symptomatic patient should undermine the effectiveness of sizing assays without DNA sequencing in the diagnosis of CALR mutations.

[Orbital mantle cell lymphoma]. / Lymphome du manteau orbitaire.

Ophthalmological involvement in mantle cell lymphoma is rare. An 84-year-old man presented with bilateral proptosis. Orbital biopsy established the diagnosis of mantle cell lymphoma. The diagnosis of mantle cell lymphoma is anatomopathological and immuno-histochemical. Orbital involvement is found most often in advanced stages of the disease, but may be the presenting sign as in the case of our patient. Treatment has improved with the use of rituximab, especially in elderly patients not eligible for autologous hematopoietic stem cell transplant.

Thrombotic microangiopathy with skin localization secondary to cytarabine-daunorubicin association: report of a case.

The thrombotic microangiopathy is a syndrome characterized by the combination of mechanical hemolytic anemia, peripheral thrombocytopenia, and organ failure of variable severity. In addition to the idiopathic form, several cases are identified as secondary to pregnancy, infections, disease systems, organ transplants, and cancer. Other forms are secondary to drugs including antimitotics. We report the case of a patient followed for acute myelogenous leukemia. She received induction chemotherapy combining daunorubicin and cytarabine, complicated by thrombotic thrombocytopenic purpura.

Bing and neel syndrome.

Introduction. We report the case of a Bing and Neel syndrome revealed by an isolated left ptosis. Case Report. a 57-year-old man was followed up since October 2003 for a typical Waldenström's macroglobulinemia. A first complete remission was obtained with chlorambucil. In August 2004, he relapsed. A second complete remission was obtained with RFC chemotherapy regimen (rituximab, fludarabine, and cyclophosphamide). In October 2009, the patient presented with an isolated left ptosis revealing a Bing and Neel syndrome. The diagnosis was suspected on MRI and confirmed by the detection in the CSF of a monoclonal IgM similar to the one found in the plasma. A quite good partial remission has been obtained after one course of RDHAP (rituximab, dexamethasone, cytarabine, and cisplatin) and 3 courses of RDHOx (rituximab, dexamethasone, cytarabine, and oxaliplatin), in addition to ten intrahectal chemotherapy injections. The treatment was followed by intensification and autologous stem cell transplantation. At D58, the patient died due to a septic shock. Conclusion. BNS is a rare and potentially treatable complication of WM. It should be considered in patients with neurologic symptoms and a history of WM.

Monoclonal gammopathies in a Moroccan military hospital.

The aim of this study was to describe biological features and aetiology of monoclonal gammopathy diagnosed during a 10-year period in the biochemistry department of the Moroccan Military Hospital Mohamed V in Rabat. The study was performed from 1 January 2000 to 31 December 2009. The records of 261 patients living in the Rabat area in which either serum protein electrophoresis and serum and/or urine immunofixation were performed at the biochemistry department of Military Instruction Hospital in Rabat were analysed. A cohort of 182 (70%) men and 79 (30%) women, the mean ± SD (range) ages were 60.21 ± 12.56 years. All patients were Caucasian. Electrophoresis found that 211 (80.84%) of the patients had a monoclonal gammopathy. Immunofixation confirmed that 251 (96.17%) patients had a monoclonal band in serum. In our cohort, MM was the most frequent diagnosis, our patients were late diagnosed.

[Blood isolates epidemiology in a clinical haematology department]. / Epidémiologie des isolats d'hémocultures: expérience d'un service d'hématologie clinique.

Cancer chemotherapy is responsible for infections by decreasing the phagocytosis and chemotaxis of polymorphonuclear. We conducted a retrospective analysis during the period from 18/10/2006 to 21/05/2008, on all bacteria isolated from blood cultures performed in the department of clinical hematology at the hospital military instruction Mohamed V. One hundred and sixty two blood isolates were selected; Gram positive cocci (CGP) accounted for 60.34% and Gram negative bacilli (GNB) for 24.14%. Coagulase negative staphylococci (SNA) and S. aureus presented a resistance to methicilline respectively 54.55% and 22.22%. Prevalence of Gram positive cocci is consistent with the results of the EORTC (International Antimicrobial Therapy Cooperative Group). Analysis of resistance patterns of all species, except for staphylococci, showed phenotypes essentially community, sometimes wild. In conclusion probabilistic antibiotic treatement of bacteraemia in the haematology department should focus among other staphylococci resistant to methicilline.

[Primary gastric lymphoma: a retrospective series of 35 cases]. / Le lymphome gastrique primitif (etude rétrospective d'une série de 35 patients).

BACKGROUND: Primary gastric non Hodgkin's lymphoma (PGNHL) is the most common site of extranodal malignant lymphoma. It is a rare subtype of malignancy, for which no consensus exists about treatment. AIM: The purpose of This paper was to assess the managemnt of PGNHL. METHODS: Between January 1992 and December 2001, 35 patients were retrospectively evaluated. RESULTS: Of the 35 patients, with a mean age of 44 years old Signs and symptoms are unspecific. 20 (57.1%) were in stage IE/IIE1. 15 patients (42.8%) were in stage IIE2,IIIE,IVE. These patients were treated with primary surgery with or without chemotherapy (11; 31.4%); primary chemotherapy (CT) alone with surgery in one patient (21; 60%) and three patients with gastric MALT lymphoma were treated by Helicobacter pylori eradication. The 5 year survival rates of the 35 patients are 62, 86%. There was no significant difference in the 5 year survival rate between the patients with low grade lymphoma and the patients with large grade lymphoma (75% versus 60%, P = 0.467). The 5-year survival rates for stage I/IIE1 and IIE2/III/IV patients were 80%, 53.3% respectively (p < 0.144). Of the 11 primary surgical groups with or without chemotherapy, the 5 year survival rate is 90.9%. Of the 21 patients who received CT, alone or combined with surgery, the survival rate is 52.4%. There was significant difference between the two groups (P = 0.05). Of the 3 patients with low-grade mucosa-associated lymphoid tissue (MALT) lymphoma with only oral anti-Helicobacter pylori regimen remained disease-free after a median follow-up of two years. CONCLUSIONS: This study suggested that primary surgical resection may be important factor predicting the long-term survival of patients with primary gastric NHL. H. pylori eradication therapy was an effective first-line treatment for patients with gastric MALT lymphoma.

[Kidney vasculitis connected to cryoglobulinemia IIA and hepatitis B]. / Vascularite rénale dans le cadre d'une cryoglobulinémie mixte associée au virus de l'hépatite B.

We report the case of a 70 years old patient hospitalized for renal insufficiency and deterioration of the general state. The electrophoresis of serum proteins on freezing of agarose reveals the presence of a discrete peak of monoclonal pace on the level of the gammaglobulines identified by serum immunofixation like IgM of the kappa type. The research of the cryoglobulinemia carried out in a laboratory of city was made positive and typified like a monoclonal cryoglobulinemia IgM kappa, thus directing the diagnosis towards a disease of Waldenström. However, the result of the biopsy medullary made exclude any lymphoprolifératif syndrome. The positivity of the serology of hepatitis B justified a second request for study of the cryoglobulinemia, carried out within our laboratory. The cryoglobuline was typified like mixed (IgM kappa monoclonal and IgG polyclonales). This result associated with the immunological assessment and the renal biopsy made retain for our patient the diagnosis of a kidney vasculitis connected to an infection chronicle by the virus of hepatitis B. This observation points out the interest of the preanalytic, analytic and post analytic phases in the study of the cryoglobulinemias. A good technical control is today the only guarantee of the quality of the result of this examination which has large importance in internal medicine in the etiologic assistance with the diagnosis of certain clinical demonstrations.

[Cutaneous blastomycosis revealing intravascular B-cell lymphoma: a case in Morocco]. / Blastomycose cutanée révélant un lymphome B intravasculaire: une observation marocaine.

Blastomycosis is a systemic fungal infection caused by a thermally dimorphic fungus, Blastomyces dermatitidis. The incidence in immunocompromised patients has increased in the last two decades. A 55-year-old man consulted for inflammatory nodules on the forearm. Biopsy of one nodules showed a pseudoepitheliomatous hyperplastic epidermis overlaying a dense agranulomatous inflammatory infiltrate containing free-formed ovoid bodies enclosing giant macrophageous cells. These findings were consistent with blastomycosis. After a month of treatment cutaneous lesions regressed partially but the patient's general status continued to deteriorate with the appearance of an edematous-ascitic syndrome and icterus. Laboratory blood testing demonstrated cholestasia and abdominal ultrasound showed hepatosplenomegaly. Needle liver biopsy revealed giant B-cell lymphomatous infiltration of the hepatic ducts. The patient's condition worsened rapidly and he died five months after diagnosis despite four rounds of chemotherapy. Blastomycosis is rare in Morocco. Primary infection is usually a pneumonic process. Isolated cutaneous infection is possible but uncommon. To our knowledge the association of blastomycosis and intravascular lymphoma has not been previously reported. In immunocompromised patients, clinical findings can be alarming and the outcome can be rapidly fatal.

[Cardiac localization of non Hodgkin's lymphoma: a study on four cases]. / Localisations cardiaques du lymphome non hodgkinien: à propos de quatre cas.

INTRODUCTION: Cardiac localization of non-Hodgkin's lymphoma is rare and usually seen in the latest phase of the disease. However, its frequency is certainly under-estimated because 20% of patients who die of lymphoma have a heart involvement at autopsy. EXEGESIS: We report four cases of cardiac involvement of non-Hodgkin's lymphoma with various revelation modalities. Features of cardiac lymphoma are multiple and not specific. Echocardiography can be helpful in finding cardiac localizations, which can involve every cardiac structure. Histological proof of such an involvement is sometimes difficult to obtain. CONCLUSION: Prognosis of cardiac lymphoma remains poor despite the real progress in the treatment of lymphomas.

[Digestive malacoplakia]. / Malacoplakie digestive.

INTRODUCTION: Malacoplakia, a chronic granulomatous disease, rarely involves the digestive tube and, when it does, takes on a pseudotumoral aspect. OBSERVATION: A 37 year-old man was hospitalized for chronic diarrhea that had progressed over 15 years. He exhibited an edematous-ascitic syndrome and bilateral pleurisy together with, biologically, a malabsorption syndrome. The endoscopic examinations (fibroscopy and colonoscopy) revealed polypoid tumor-like formations. An image of tumoral stenosis of the sigmoid-colic junction was revealed on barite lavage. Histological examination of the surgical sample (wide left colectomy of one third of the transverse) was suggestive of malacoplakia (histiocytes with characteristic Michaelis-Gutmann bodies). DISCUSSION: Malacoplakia is an inflammatory disease predominantly affecting the urogenital tract. Other organs can be involved. Its clinical symptomatology is highly disparate. The interest of this disease is in its pathogenesis, on which, in fact, its treatment is based.

[What is new in antithrombotic treatment?]. / Quoi de neuf dans le traitement antithrombotique?

Real progresses have been made during the past years in the comprehension of hemostasis mechanisms, along with rising of new antithrombotic drugs. The later include: 1) direct inhibitors of thrombin such as hirudin, bivalirudin, argatroban, melagatran and ximelagatran; 2) inhibitors of factor Xa such as the synthetic pentasaccharid and DX-9065a; 3) inhibitors of factor IXa; 4) inhibitors of tissue factor-factor VIIa complex such as tissue factor pathway inhibitor (TFPI) or NAPc2 (nematode anticoagulant peptide); 5) drugs enhancing endogenous anticoagulant activity, such as protein C or activated protein C; 6) drugs modulating endogenous fibrinolytic activity. These new drugs are promising a real decrease in mortality and morbidity due to venous thrombo-embolic disease, which is considered as a public health issue. Both physicians and biologists are concerned by these new antithrombotic agents, the former to think about new treatment strategies, the later to monitore, if necessary, the effects of such new drugs. Our review does not include antiplatelet agents which are indicated only in arterial thrombosis.