Microgranular variant APL

Autor*: A. Buliková, J. Kissová

Description:
42 years old man comes to hospital with 2 weeks long history of fever and 1 weeks history of bleeding symptoms. Laboratory evaluation shows marked leukocytosis (95G/l), mild anaemia (121 g/l) and moderate thrombocytopenia (45G/l) in CBC and coagulopathy. Peripheral blood smears show hypogranular or agranular pathological promyelocytes, but some Auer rods occur. Similar picture show bone marrow smears but some cells contain bundles of Auer rods or occasionally even faggot cells is find. Strong positivity of myeloperoxidase staining supports diagnose of promyelocytic leukaemia (not confirmed by cytogenetic or molecular genetic evaluation in case of patient's really short survivor).

Zařazení*:
Acute promyelocytic leukaemia (AML with t(15;17)(q22;q12), (PML/RAR alfa) and variants)

Přílohy*:

microgranular variant APL-PB
The main feature of this type acute myeloblastic leukaemia is bi-lobed nuclear shape and presence of dust-like reddish granules in cytoplasm. Some tumour cells can be even agranular. Leukocytosis is usual when one compares this type with classic hyper-granular form.   / Hlavním rysem tohoto typu akutní myeloblastické leukémie je bilobární tvar jádra a přítomnost prachových načervenalých granulí v cytoplazmě. Některé nádorové buňky mohou být dokonce agranulární. Leukocytóza je obvykla v porovnání s klasickou hypergranulární variantou. Description: The main feature of this type acute myeloblastic leukaemia is bi-lobed nuclear shape and presence of dust-like reddish granules in cytoplasm. Some tumour cells can be even agranular. Leukocytosis is usual when one compares this type with classic hyper-granular form.
Type: peripheral blood
Klíčová slova*: promyelocyte, tumour cell
microgranular variant APL- PB
Some pathological promyelocytes are completely agranular (lower left) with kidney shaped nucleus. Some Auer rods even in bundles can be find almost in every cases - in this one there are 3-4 thick Auer rods in the second cell from bellow at right.   / Některé patologické promyelocyty jsou úplně agranulární (vlevo dole) s ledvinovým tvarem jádra. Auerovy tyče  dokonce ve snopcích mohou být nalezeny téměř ve všech případech - v tomto jsou 3-4 silné Auerovy tyče ve druhé buňce zespodu vpravo. Description: Some pathological promyelocytes are completely agranular (lower left) with kidney shaped nucleus. Some Auer rods even in bundles can be find almost in every cases - in this one there are 3-4 thick Auer rods in the second cell from bellow at right.
Type: peripheral blood
Klíčová slova*: promyelocyte, Auer rod
microgranular variant APL- PB
Tumour cell with bi-lobar nucleus, clear nucleoli in every nuclear part, some (upper left) or no (lower right) cytoplasmic granulation.  / Nádorové buňky s bilobárním jádrem, jasnými jadérky v každé části jádra, nějakou (vlevo nahoře) nebo žádnou (vpravo dole) cytoplazmatickou granulací. Description: Tumour cell with bi-lobar nucleus, clear nucleoli in every nuclear part, some (upper left) or no (lower right) cytoplasmic granulation.
Type: peripheral blood
Klíčová slova*: promyelocyte, tumour cell
microgranular variant APL- PB
Bi-lobar nucleus and clear Auer rod but in almost agranular cytoplasm are typical features in the cell at upper left. Two large lobes of nucleus joined by a narrow bridge (cell at the 3 o'clock) present another characteristics of the variant form PML.  / Bilobární jádro a  jasná Auerova tyč, ale v téměř agranulární cytoplazmě,  jsou typické známky v buňce nahoře vlevo. Dva velké laloky jádra spojené tenkým můstkem (buňka na 3. hodině) představují jiné charakteristické rysy variantní formy PML. Description: Bi-lobar nucleus and clear Auer rod but in almost agranular cytoplasm are typical features in the cell at upper left. Two large lobes of nucleus joined by a narrow bridge (cell at the 3 o'clock) present another characteristics of the variant form PML.
Type: peripheral blood
Klíčová slova*: promyelocyte
microgranular variant APL- PB
Numerous bi-lobar or kidney shaped nuclei and agranular or fine granular cytoplasm in "promyelocytes" in peripheral blood smears in patient with AML M3v. Cytoplasm of some cells (example at upper left) can contain more pronounced usually isolated red inclusion.  / Početná bilobární či ledvinovitá jádra a agranulární či jemně granulovavaná cytoplazma v "promyelocytech" nátěru periferního krvi u pacienta s AML M3v. Cytoplazma některých buněk (příklad vlevo nahoře) mohou obsahovat více vyjádřené obvykle izolované červené inkluze. Description: Numerous bi-lobar or kidney shaped nuclei and agranular or fine granular cytoplasm in "promyelocytes" in peripheral blood smears in patient with AML M3v. Cytoplasm of some cells (example at upper left) can contain more pronounced usually isolated red inclusion.
Type: peripheral blood
Klíčová slova*: promyelocyte
microgranular variant APL- BM
Bi-lobular or multilobular (cell at the middle right) nuclei and fine reddish granulation in cytoplasm are also typical in tumour cells in  bone marrow.  / Bilobární nebo multilobulární (buňka ve středu vpravo) jádra a jemná načervenalá granulace v cytoplazmy jsou typické taktéž v nádorových buňkách kostní dřeně. Description: Bi-lobular or multilobular (cell at the middle right) nuclei and fine reddish granulation in cytoplasm are also typical in tumour cells in bone marrow.
Type: bone marrow
Klíčová slova*: promyelocyte
microgranular variant APL- BM
In all cases of acute leukaemia with bilobar nuclei it is really necessary carefully search for cells with bundles Auer rods in cytoplasm ("faggot cells"). This finding brings high suspicion to diagnose of microgranular variant of APL, which should be confirmed by cytogenetic and/or molecular genetic examination - presence of 
t(15;17) and/or PML/RARalfa fusion gene.    / Ve všech případech akutní leukémie s bilobárními jádry je opravdu nezbytné pečlivě pátrat po buňkách se snopci Auerových tyčí v cytoplazmě ("faggot cells"). Tento nález přináší vysoké podezření na diagnózu mikrogranulární varianty APL, která by měla být potvrzena cytogenatickým a/nebo molekulárně genatickým vyšetřením - přítomnost t(15;17) a/nebo PML/RARalfa fúzního genu. Description: In all cases of acute leukaemia with bilobar nuclei it is really necessary carefully search for cells with bundles Auer rods in cytoplasm ("faggot cells"). This finding brings high suspicion to diagnose of microgranular variant of APL, which should be confirmed by cytogenetic and/or molecular genetic examination - presence of t(15;17) and/or PML/RARalfa fusion gene.
Type: bone marrow
Klíčová slova*: promyelocyte, Auer rod, faggot cell
microgranular variant APL- BM
Bi-lobar or irregular nuclear shape in variant PML. There is one faggot cell at the upper left. Some tumour cells in variant APL can have cytoplasmic protrusions (four from five cells in lower part of the picture)-   / Bilobární nebo nepravidelný tvar jádra u variantní PML. Jedna "faggot cell" je nahoře vlevo. Některé nádorové buňky u variantní APL mohou mít cytoplazmatické výběžky (čtyři z pěti buněk v dolní části obrázku).  Description: Bi-lobar or irregular nuclear shape in variant PML. There is one faggot cell at the upper left. Some tumour cells in variant APL can have cytoplasmic protrusions (four from five cells in lower part of the picture)-
Type:
Klíčová slova*: faggot cell
microgranular variant APL- BM
Four tumour cells in variant APL with bi-lobar nuclei, rather fine cytoplasmic granulation and cytoplasmic projections at the outline of the cell.  There is one typical hypergranular promyelocyte at the upper left with cytoplasm filed by large numerous azurophilic granules, which totally obscure the nuclear cytoplasmic margin.  / Čtyři nádorové buňky u varinatní APL s bilobárními jádry, spíše jemnou cytoplazmatickou granulací a cytoplazmatickými výběžky na zevním obvodu buňky. Jeden typický hypergranulární promyelocyt je nahoře vlevo s cytoplazmou naplněnou velkými početnými azurofilními granuly, která úplně zakrývají rozhraní mezi jádrem a cytoplazmou. Description: Four tumour cells in variant APL with bi-lobar nuclei, rather fine cytoplasmic granulation and cytoplasmic projections at the outline of the cell. There is one typical hypergranular promyelocyte at the upper left with cytoplasm filed by large numerous azurophilic granules, which totally obscure the nuclear cytoplasmic margin.
Type: bone marrow
Klíčová slova*: promyelocyte
microgranular variant APL- BM, MPOX
Myeloperoxidase reaction is strongly positive in contrast with "monocyte appearance" of some tumour cells in variant APL (in monocytes is usually weak or almost negative MPOX reaction). This finding can support suspicion to granulocytic origin of leukemic cells.   / Reakce myeloperoxidázy je silně pozitivní na rozdíl od "monocytoidního vzhledu" některých nádorových buněk u variantní APL (v monocytech je obvykle slabá nebo téměř negativní reakce MPOX). Tento nález může podpořit podezření na granulocytární původ leukemických buněk. Description: Myeloperoxidase reaction is strongly positive in contrast with "monocyte appearance" of some tumour cells in variant APL (in monocytes is usually weak or almost negative MPOX reaction). This finding can support suspicion to granulocytic origin of leukemic cells.
Type: bone marrow
Klíčová slova*: myeloperoxidase stain